Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Hospitalised adolescents with a heart murmur generally fall into two major groups, those who are there for investigation of a heart murmur that has been recently found or those that have had a positive diagnosis and are amenable and suitable for treatment. (Mahnke, C. B et al 2004).

Acute rheumatic fever (ARF) results from the body’s autoimmune response to a throat infection caused by Streptococcus pyogenes, also known as the group A Streptococcus bacteria. Rheumatic heart disease (RHD) refers to the long-term cardiac damage caused by either a single severe episode or multiple recurrent episodes of ARF. It is RHD that remains a significant worldwide cause of morbidity and mortality, particularly in resource-poor settings. While ARF and RHD were once common across all populations, improved living conditions and widespread treatment of superficial S. pyogenes infections have caused these diseases to become comparatively rare in wealthy areas (Carapetis, 2007). Currently, these diseases mainly affect those in low- and middle-income nations, as well as in indigenous populations in wealthy nations where initial S. pyogenes infections may not be treated, which allows for the development of harmful post-infectious sequelae (Carapetis, 2007).Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

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The development of ARF occurs approximately two weeks after S. pyogenes infection (Gewitz, et al., 2015). The clinical manifestations and symptoms of ARF can be severe and are described in the Revised Jones Criteria (Gewitz, et al., 2015). Symptoms of ARF can include polyarthritis, carditis, chorea, the appearance of subcutaneous nodules, and erythema marginatum or a rash associated with ARF (Gewitz, et al., 2015; Martin, et al., 2015). These symptoms usually require patients to be hospitalized for two to three weeks, during which time the outward symptoms resolve, but the resultant cardiac damage may persist. With repeated S. pyogenes pharyngitis infections, ARF can recur and cause cumulative damage to the heart valves (Martin, et al., 2015).

Heart failure (congestive) is caused by many conditions including coronary artery disease, heart attack, cardiomyopathy, and conditions that overwork the heart. Symptoms of heart failure include

congested lungs,
fluid and water retention,
dizziness,
fatigue and weakness, and
rapid or irregular heartbeats.

There are two types of congestive heart failure, systolic or left-sided heart failure; and diastolic or right-sided heart failure. Treatment, prognosis, and life-expectancy for a person with congestive heart failure depends upon the stage of the disease.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

This chapter will briefly cover the epidemiology and pathophysiology of ARF and RHD, and will also outline the clinical manifestations, diagnostic considerations, and recommended treatment and management options for both conditions. Finally this chapter will also highlight prevention strategies for ARF and RHD and will discuss current vaccination efforts against S. pyogenes.

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Epidemiology of ARF and RHD

Burden of Disease

The global burden of ARF and RHD is significant, and is predominantly found in populations living in low-resource settings (Carapetis, Steer, Mulholland, & Weber, 2005). Incidence rates of ARF are poorly documented in most low- and middle-income countries, including in populations with a high prevalence of RHD, where it is presumed that a high incidence of ARF also occurs. This relates both to the lack of infrastructure for disease surveillance in those settings, but also to a paucity of ARF cases that are presented for clinical care. It is not known if the latter issue is a result of health-seeking behavior (people with ARF who choose not to seek health care), or due to inadequate diagnosis of ARF by health staff. The latter may in turn be due to true misdiagnosis as a result of problems with training, a lack of access to diagnostic facilities (such as electrocardiography, streptococcal serology, acute phase reactant testing, and echocardiography); or possibly because many cases of ARF may be milder, or even sub-clinical, in highly endemic settings (Bishop, Currie, Carapetis, & Kilburn, 1996).

Coarctation of the aorta is a congenital (present at birth) heart defect involving a narrowing of the aorta.

The aorta is the large artery that carries oxygen-rich (red) blood from the left ventricle to the body. It is shaped like a candy cane. The first section moves up towards the head (ascending aorta), then curves in a C-shape as smaller arteries that are attached to it carry blood to the head and arms (aortic arch). After the curve, the aorta becomes straight again, and moves downward towards the abdomen, carrying blood to the lower part of the body (descending aorta).

The narrowed segment called coarctation can occur anywhere in the aorta, but is most likely to happen in the segment just after the aortic arch. This narrowing restricts the amount of oxygen-rich (red) blood that can travel to the lower part of the body. Varying degrees of narrowing can occur.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Anatomy of a heart with a coarctation of the aorta
Click Image to Enlarge
The more severe the narrowing, the more symptomatic a child will be, and the earlier the problem will be noticed. In some cases, coarctation is noted in infancy. In others, however, it may not be noted until school-age or adolescence.

About one-half of children with coarctation of the aorta also have a bicuspid aortic valve—a valve that has 2 leaflets instead of the usual 3.

Coarctation of the aorta occurs in a small percentage of children with congenital heart disease. Boys have the defect more commonly than girls do.

What causes coarctation of the aorta?
Some congenital heart defects may have a genetic link that causes heart problems to occur more often in certain families. Most of the time this heart defect occurs sporadically (by chance), with no clear reason for its development.

Why is coarctation a concern?
Coarctation of the aorta causes several problems:

The left ventricle has to work harder to try to move blood through the narrowing in the aorta. Eventually, the left ventricle is no longer able to handle the extra workload, and it fails to pump blood to the body efficiently.

Blood pressure is higher before the narrowing, and lower past the narrowing. Older children may have headaches from too much pressure in the vessels in the head, or cramps in the legs or abdomen from too little blood flow in that region. Also, the kidneys may not make enough urine since they require a certain amount of blood flow and a certain blood pressure to perform this task.

The walls of the ascending aorta, the aortic arch, or any of the arteries in the head and arms may become weakened by high pressure. Spontaneous tears in any of these arteries can occur, which can cause a stroke or uncontrollable bleeding.

What are the symptoms of coarctation of the aorta?
Symptoms noted in early infancy are caused by moderate to severe aortic narrowing. The following are the most common symptoms of coarctation of the aorta. However, each child may experience symptoms differently. Symptoms may include:

Irritability

Pale skin

Sweating

Heavy and/or rapid breathing

Poor feeding

Poor weight gain

Cold feet and/or legs

Diminished or absent pulses in the feet

Blood pressure in the arms significantly greater than the blood pressure in the legs

Mild narrowing may not cause symptoms at all. Often, a school-aged child or adolescent is simply noted to have high blood pressure or a heart murmur during a physical exam. Some may complain of headaches or cramps in the lower sections of the body.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

The symptoms of coarctation of the aorta may resemble other medical conditions or heart problems. Always consult your child’s health care provider for a diagnosis.

How is coarctation of the aorta diagnosed?
Your child’s health care provider may have heard a heart murmur during a physical exam, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the obstruction in the coarctation segment of the aorta. Your child’s symptoms will also help with the diagnosis.

A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical exam, listen to your child’s heart and lungs, and make other observations that help in the diagnosis. The location within the chest that the murmur is heard best, as well as the loudness and quality of the murmur (harsh, blowing, etc.) will give the cardiologist an initial idea of which heart problem your child may have. Diagnostic testing for congenital heart disease varies by the child’s age, clinical condition, and institutional preferences. Some tests that may be recommended include the following:

Chest X-ray. Diagnostic test which uses invisible X-ray energy beams to produce images of internal tissues, bones, and organs onto film.

Electrocardiogram (ECG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias), and detects heart muscle damage.

Echocardiogram (echo). A procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves. The vast majority of aortic coarctations are diagnosed by echocardiography.

Cardiac catheterization (cath). A diagnostic procedure that uses threading a catheter through the arteries and veins of the groin and advancing this catheter up to the heart. Dye is squirted into the heart and aorta and pictures are taken of the anatomy. Catheterization may also be used to improve the coarctation if the child is big enough.

Magnetic resonance imaging (MRI). A diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.

Treatment for coarctation of the aorta
Specific treatment for coarctation of the aorta will be determined by your child’s health care provider based on:

Your child’s age, overall health, and medical history

Extent of the disease

Your child’s tolerance for specific medications, procedures, or therapies

Expectations for the course of the defect

Your opinion or preference

Coarctation of the aorta is treated with repair of the narrowed vessel. Several options are currently available.

Interventional cardiac catheterization. Cardiac catheterization may be an option for treatment. During the procedure, the child is sedated and a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin and guided to the inside of the heart. Once the catheter is in the heart, the cardiologist will pass an inflated balloon through the narrowed section of the aorta to stretch the area open. A small device, called a stent, may also be placed in the narrowed area after the balloon dilation to keep the aorta open. Overnight observation in the hospital is generally required.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Surgical repair. Your child’s coarctation of the aorta may be repaired surgically in an operating room. The surgical repair is done under general anesthesia. The narrowed area is either surgically removed, or made larger with the help of surrounding structures or a patch.

Some infants will be very sick, requiring care in the intensive care unit (ICU) prior to the procedure, and could possibly even need emergency repair of the coarctation. Others, who have few symptoms, will have the repair scheduled on a less urgent basis.

A vascular ring is a type of congenital lesion where normal vessels are in an abnormal location and may cause compression or obstruction of the esophagus or airway.

Cardiology (from Greek καρδίᾱ kardiā, “heart” and -λογία -logia, “study”) is a branch of medicine that deals with the disorders of the heart as well as some parts of the circulatory system. The field includes medical diagnosis and treatment of congenital heart defects, coronary artery disease, heart failure, valvular heart disease and electrophysiology. Physicians who specialize in this field of medicine are called cardiologists, a specialty of internal medicine. Pediatric cardiologists are pediatricians who specialize in cardiology. Physicians who specialize in cardiac surgery are called cardiothoracic surgeons or cardiac surgeons, a specialty of general surgery.

Although the cardiovascular system is inextricably linked to blood, cardiology is relatively unconcerned with hematology and its diseases. Some obvious exceptions that affect the function of the heart would be blood tests (electrolytedisturbances, troponins), decreased oxygen carrying capacity (anemia, hypovolemic shock), and coagulopathies.

Physical examination of the cardiovascular system is central to contemporary teaching and practice in clinical medicine. Evidence about its value focuses on its diagnostic accuracy and varies widely in methodological quality and statistical power. This makes collation, analysis, and understanding of results difficult and limits their application to daily clinical practice. Specific factors affecting interpretation and clinical application include poor standardisation of observers’ technique and training, the study of single signs rather than multiple signs or signs in combination with symptoms, and the tendency to compare physical examination directly with technological aids to diagnosis rather than explore diagnostic strategies that combine both. Other potential aspects of the value of physical examination, such as cost effectiveness or patients’ perceptions, are poorly studied. This review summarises the evidence for the clinical value of physical examination of the cardiovascular system. The best was judged to relate to the detection and evaluation of valvular heart disease, the diagnosis and treatment of heart failure, the jugular venous pulse in the assessment of central venous pressure, and the detection of atrial fibrillation, peripheral arterial disease, impaired perfusion, and aortic and carotid disease. Although technological aids to diagnosis are likely to become even more widely available at the point of care, the evidence suggests that further research into the value of physical examination of the cardiovascular system is needed, particularly in low resource settings and as a potential means of limiting inappropriate overuse of technological aids to diagnosis.

These arteries may be those carrying blood to the body, to the lungs, or both. They are most easily broken into three main groups: vascular rings, the innominate compression syndrome, and pulmonary arterial slings.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Vascular rings include a number of anatomic variations of abnormal development of the aortic arch complex resulting in the formation of a ring encircling both the trachea and esophagus.

The aorta originally develops as a series of arches with bilateral symmetry. By the end of the second month of fetal development, parts of the arch complex have regressed leaving the “typical” anatomy of a left aortic arch with three arch branches (innominate, left common carotid and left subclavian) and a left-sided ductus arteriosus from the proximal left pulmonary artery to the aorta in the general vicinity of the left subclavian artery origin.

Virtually all vascular rings can be explained by abnormal regression or persistence of different components of the bilateral aortic arch complex.

The two most common anatomic variants of true vascular rings, occurring in nearly equal frequency, are persistent double aortic arch and right aortic arch with anomalous origin of the left subclavian artery.

In the latter, the left-sided ligamentum arteriosum completes the vascular ring as it passes from the left pulmonary artery to the left subclavian artery as it travels abnormally behind the esophagus.

Rheumatic heart disease is the most serious complication of rheumatic fever. Acute rheumatic fever follows 0.3% of cases of group A beta-hemolytic streptococcal pharyngitis in children. As many as 39% of patients with acute rheumatic fever may develop varying degrees of pancarditis with associated valve insufficiency, heart failure, pericarditis, and even death. With chronic rheumatic heart disease, patients develop mitral valve stenosis with varying degrees of regurgitation, atrial dilation, arrhythmias, and ventricular dysfunction. Chronic rheumatic heart disease remains the leading cause of mitral valve stenosis and valve replacement in adults in the United States.

Acute rheumatic fever and rheumatic heart disease are thought to result from an autoimmune response, but the exact pathogenesis remains unclear. Although rheumatic heart disease was the leading cause of death 100 years ago in people aged 5-20 years in the United States, incidence of this disease has decreased in developed countries, and the mortality rate has dropped to just above 0% since the 1960s

After surgery, infants will return to the intensive care unit ICU to be closely monitored during recovery.

While your child is in the ICU, special equipment will be used to help him or her recover, and may include the following:

Ventilator. A machine that helps your child breathe while he or she is under anesthesia during the operation. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while he or she is too sleepy to breathe effectively on his or her own. Many children remain on the ventilator for a while after surgery so they can rest.

Intravenous (IV) catheters. A small, plastic tube is inserted through the skin into blood vessels to provide IV fluids and important medicines that help your child recover from the operation.

Arterial line. A specialized IV placed in the wrist, or other area of the body where a pulse can be felt, that measures blood pressure continuously during surgery and while your child is in the ICU.

Nasogastric (NG) tube. A small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery.

Urinary catheter. A small, flexible tube that allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. After surgery, the heart will be a little weaker than it was before, and the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys remove excess fluid from the body.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Etiology

Rheumatic heart disease results from either single or repeated attacks of rheumatic fever that results in rigidity and deformity of valve cusps, the fusion of the commissures, or shortening and fusion of the chordae tendineae. Over 2 to 3 decades, valvular stenosis and/or regurgitation results. In chronic rheumatic heart disease, the mitral valve alone is the most commonly affected valve in an estimated 50 to 60% of cases. Combined lesions of both the aortic and mitral valves occur in 20% of cases. Involvement of the tricuspid valve occurs in about 10% of cases but only in association with mitral or aortic disease. Tricuspid valve cases are thought to be more common when recurrent infections have occurred. The pulmonary valve is rarely affected.

Epidemiology

Rheumatic heart disease (RHD) is the most critical form of acquired heart disease in children and young adults living in developing countries. RHD accounts for approximately 15 to 20 percent of all patients with heart failure in endemic countries.[2]

A study of rheumatic heart disease cases estimated that in 2015, there were globally 33.4 million cases of RHD, 10.5 million disability-adjusted life-years due to RHD, and 319400 deaths due to RHD.[3] The incidence of rheumatic heart disease is highest in Oceania, central sub-Saharan Africa, and South Asia. In 2015, there was noted to be 3.4 cases per 100,000 population in nonendemic countries and 444 cases per 100,000 population in endemic countries.[3]

Rheumatic heart disease affects predominantly those living in poverty with inadequate access to health care and unchecked exposure to group A streptococcus. A systematic review and meta-analysis calculated the prevalence of clinically silent RHD (21.1 per 1000 people) to be approximately seven to eight times higher than that of clinically manifest disease (2.7 per 1000 people). Prevalence of rheumatic heart disease increases with age, from 4.7 per 1000 children at 5 years of age to 21.0 per 1000 children at 16 years of age.[4] Based on this data, estimates are that the RHD burden could increase by as much as double than that in the Global Burden of Disease study. Based on the fact that children in sub-Saharan Africa represent 6 to 7 percent of total global RHD burden, there may be an estimated 50 to 80 million persons currently affected with RHD worldwide.[5]

Pathophysiology

Rheumatic heart disease is the result of valvular damage caused by an abnormal immune response to Streptococcus pyogenes infection, which is classified as a group A streptococcus that causes acute rheumatic fever.[6]. Acute rheumatic fever occurs around three weeks after group A streptococcal pharyngitis that can affect joints, skin, brain, and heart.[7] After multiple episodes of rheumatic fever, progressive fibrosis of heart valves can occur, which can lead to rheumatic valvular heart disease. If valvular heart disease remains untreated, then heart failure or death may occur. The precise pathophysiology is not well known Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

History and Physical

Rheumatic fever is the primary cause of acquired heart disease in children and young adults worldwide. Rheumatic fever occurs 2 to 3 weeks after a group A beta-hemolytic streptococcal pharyngeal infection.[8]

Carditis is the most serious presentation of rheumatic fever. The symptoms and signs of carditis are dependent on the areas of the heart involved, which includes pericardium, myocardium or heart valves. The presentation of a pericardial friction rub on auscultation leans toward the diagnosis of pericarditis. The presence of signs of congestive heart failure points toward a diagnosis of myocarditis, which includes but not limited to lower extremity edema, shortness of breath with exertion or rest, abdominal distension, or inability to lay flat due to shortness of breath (orthopnea). Myocarditis in the absence of valvular disease is unlikely to be rheumatic in origin. Therefore, an apical systolic or basal diastolic murmur should be auscultated on physical exam. Mitral regurgitation is the most common valvular lesion, which is an apical pan-systolic murmur on auscultation.[8] Aortic regurgitation is less common. If patients have a known history of rheumatic heart disease, a change in the character of the murmur or presence of a new murmur on auscultation leads to the diagnosis of acute rheumatic heart fever. Rheumatic heart disease predominantly affects the left-sided cardiac valves.[8] The tricuspid valve and rarely pulmonary valve can be affected, but very unlikely without mitral valve involvement.

Rheumatic heart disease has a variety of clinical manifestations including myocarditis, decompensated congestive heart failure, arrhythmias (i.e., atrial fibrillation), and valvular heart disease. Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Myocarditis can result in conduction disturbances in the heart. Therefore, an EKG is necessary. An EKG can show varying forms of heart block including first degree, second degree or third degree AV block.While rheumatic heart disease (RHD) may develop after a single bout of acute rheumatic fever (ARF), it is typically associated with recurrent episodes of ARF. ARF usually occurs during childhood between the ages of 5 and 15 years. RHD can damage any part of the heart including the valves, the lining of the heart or the heart muscle, but more often damages the heart valves, especially those on the left side of the heart.

Stopping episodes of recurrent ARF can prevent rheumatic heart disease. ARF is caused by infection with the Group A Streptococcus (GAS) bacterium. This usually involves GAS infection in the throat (pharyngitis) or ‘strep throat’. There is ongoing debate about whether ARF may also occur with a Group A Streptococcus skin infection (impetigo, pyoderma).

Rheumatic heart disease (RHD) is common in remote Aboriginal communities

In Australia, the prevalence of RHD is highest in remote Aboriginal and Torres Strait Islander communities. The risk of death from RHD in these communities is 20 times that of Australians in the general population. Also at higher risk are Maori and Pacific Island peoples and those who have migrated to Australia from low to middle income countries where ARF and RHD remain widespread.

Risk factors of RHD

Risk factors include poverty, overcrowding and reduced access to medical care. Stopping episodes of recurrent ARF can prevent rheumatic heart disease. Once acute rheumatic fever is diagnosed, stopping further episodes of ARF can halt progression of the disease. Treatment can manage symptoms and reduce the risk of complications.While rheumatic heart disease (RHD) may develop after a single bout of acute rheumatic fever (ARF), it is typically associated with recurrent episodes of ARF. ARF usually occurs during childhood between the ages of 5 and 15 years. RHD can damage any part of the heart including the valves, the lining of the heart or the heart muscle, but more often damages the heart valves, especially those on the left side of the heart.

Rheumatic heart disease (RHD) is common in remote Aboriginal communities

Risk factors of RHD

Symptoms of RHD

RHD does not always cause symptoms. When it does, symptoms may include:Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Chest pain
Heart palpitations
Breathlessness on exertion
Breathing problems when lying down (orthopnoea)
Waking from sleep with the need to sit or stand up (paroxysmal nocturnal dyspnoea)
Swelling (oedema)
Fainting (syncope)
Stroke
Fever associated with infection of damaged heart valves.

Rheumatic fever explained

Without antibiotic treatment, throat infection from Group A Streptococcus can cause acute rhematic fever. In susceptible people, an immune response occurs two to three weeks following an untreated Group A Streptococcus throat infection. This response can target the brain, skin, joints and heart, and can cause inflammation.

RHD affects heart valves

The heart is a double pump with four chambers. Each chamber is sealed with a valve. The valves open and close in one direction only, so that the blood cannot flow backwards.

RHD often involves damage to the heart valves. Typically, the damaged heart valve cannot open or shut properly. This interferes with the proper flow of blood through the heart. Without treatment, the damaged valve may continue to deteriorate.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

The report by Li and colleagues in this issue of Heart once again reminds both basic scientists and clinicians of the unsolved mystery of the pathogenetic mechanism(s) responsible for the development of rheumatic fever and rheumatic heart disease.¹ These authors present observations implying a role for this virus in what they term “chronic, acquired valvar disease”. While their report evokes questions about study design, methodology, and the relative paucity of firmly supporting data, nevertheless the concept must provoke thought. Li and colleagues’ attempt to demonstrate a pathogenetic role for herpes simplex I as an agent in the development of rheumatic valvar heart disease falls short of establishing a relation. Additionally, the authors fail to adequately describe and take into account the sizeable body of evidence supporting the role for the group A β haemolytic streptococcus (Streptococcus pyogenes). Rheumatic fever and rheumatic heart disease remain a significant cause of cardiovascular morbidity and mortality in countries around the globe even into the 21st century; it is a medical and public health problem which needs a solution.

This hypothesis is not the first time that a virus has been postulated to be pathogenetically related to rheumatic fever and its sequel, rheumatic heart disease. For example, among the many previous attempts to attribute rheumatic heart disease to viral infection were the reports in the 1960s by the late George Burch and colleagues who described valvar lesions associated with coxsackie B virus infection in cynomolgus monkeys. They published intriguing photographs of mitral valve lesions in monkeys that were considered to be essentially identical to the early valvar lesions of acute rheumatic fever.^2–⁴ Twenty years later, Wedum—and later Peter Rowe—was enthusiastic about a viral contribution to rheumatic fever when she hypothesised a pathogenetic role for the measles virus in rheumatic heart disease, either alone or as a co-factor with group A streptococci.^5,⁶ Numerous other more recent examples could be cited. Yet, similar to the present report, all still remain hypotheses.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

PATHOGENESIS OF RHEUMATIC FEVER

Extensive reviews have been written about the pathogenesis of rheumatic fever and existing data have been exhaustively reviewed. The data supporting a role for the group A streptococcus as the triggering agent for development of rheumatic fever cannot be ignored.⁷ However, in concluding that currently available data are not sufficiently convincing about a role for viruses in the pathogenesis of rheumatic fever, one must be careful not to be intolerant of new concepts. It is clear that viruses may cause heart disease; viruses have been implicated in other forms of cardiovascular disease such as myocarditis and even atherosclerotic lesions to name only two.^8,⁹ Autoimmune mechanisms have been postulated to account for cardiac damage.^10,¹¹ But there is little to directly associate these viruses with rheumatic fever.

Historically there have been three major categories of hypotheses which have been promoted during the past five decades to explain a streptococcal pathogenesis for rheumatic fever. These include: (1) direct infection (for example, by the group A streptococcus); (2) effects of a streptococcal toxin (streptolysin O has been among the most commonly discussed); and (3) most feasibly, the concept of antigenic mimicry in association with an abnormal immune response.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

During the past half century, it is the concept of antigenic mimicry and/or an abnormal immune response to group A streptococcal extracellular or somatic antigens which has been most interesting. The issue was concisely summarised in a recent review by Cunningham who commented: “The disease is autoimmune in nature and most likely results in part from the production of autoreactive antibodies.”⁷ Yet, although many candidate group A streptococcal antigenic moieties have been investigated, none has been unequivocally demonstrated to be the inciting “culprit” or to fully explain the disease process.

AUTOIMMUNE HYPOTHESIS

Among the most widely promoted concepts in support of an autoimmune hypothesis involving the group A streptococcus has been the observation offered in the mid 1960s by Stollerman. Perceptively, he noted an intriguing epidemiological correlation between outbreaks of group A streptococcal upper respiratory tract infections associated with a relatively limited number of M-protein types which were followed by outbreaks of rheumatic fever. In attempting to focus on an inciting factor, he used the term “rheumatogenecity” or “rheumatogenic M-types” to try to limit the number of streptococcal strains that might have the capacity to initiate the postulated immune response during the latent period between infection and onset of clinical disease. Reports such as the one by Kuttner and Krumweide more than 60 years ago,¹² and other examples of an increased frequency with which a relatively limited number of specific M-types (for example, M-5, M-6, M-18) have been isolated during rheumatic fever outbreaks in communities, have provided support for a concept of enhanced rheumatogenecity and—by inference—suggesting antigenic mimicry. Yet, no investigation to date has incontrovertibly identified a/the “rheumatogenic factor”. This lack of evidence has resulted in scepticism (perhaps healthy) about differences in rheumatogenecity among the more than 130 now recognised different M protein types of group A streptococci and has led some investigators to search for other inciting agents.

Multiple attempts to strengthen the available data explaining a streptococcal pathogenesis by identifying a specific animal model for the study of rheumatic fever and rheumatic heart disease have not been entirely successful. Numerous group A streptococcal somatic and extracellular antigens have been injected into multiple animal species and almost always have resulted in lesions, but none of the models have the combined clinical or pathologic features of rheumatic fever/rheumatic heart disease. The only recognised natural host (and reservoir) for group A streptococci is the human, and an appropriate animal model has not been identified in half a century. Thus, laboratory efforts to define a comprehensive concept of streptococcal rheumatogenecity have been severely hampered.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

MITRAL VALVE INVOLVEMENT

An equally unsettling example when thinking about group A streptococci and antigenic mimicry is the fact that clinical studies have emphasised the overwhelming predisposition for involvement of the mitral valve. Embryologists remind us that all four valves develop from the same embryonic cell rest. If true antigenic mimicry involving heart valve tissue is responsible, would one not expect random involvement of and damage to all four heart valves in a random fashion? This observation must be answered before there can be unqualified acceptance for the antigenic mimicry hypothesis?

To omit the role of human host genetic or acquired variability in rheumatic fever susceptibility would be unwise. The concept of differing human susceptibility and human host factors was not addressed by Li and colleagues.¹ That there is variation in how humans respond to group A streptococcal antigens has been recognised for many years. Despite clinical observations such as that by Pickles¹³ in the early 1940s, and numerous molecular based studies of the latter part of the 20th and 21st century, clinicians, epidemiologists, and basic scientists have been unable to adequately explain this influence. Studies such as those of Greenberg and colleagues leave little doubt about variation in the response to group A streptococcal antigens.¹⁴ Yet, many unknowns remain to be explained. For example, conceptually it has never been explained how five individual humans infected with the same—possibly pathogenetic—strain of group A streptococci respond differently to the antigenic challenge, unless unique host factors are also considered? One of the five might have valvar involvement (carditis), another only arthritis (joints), a third chorea (central nervous system involvement), a fourth erythema marginatum (skin), and the fifth individual not develop any sequelae? What other interpretation is logical?

Even based upon the currently available incomplete data, I believe that there is little question about the pathogenetic role of the group A streptococcus in rheumatic fever! But until an encompassing prospective effort among epidemiologists, clinicians, microbiologists, immunologists, and other basic scientists can be initiated to provide more convincing data, speculation about the pathogenetic mechanism(s) will persist. As clinicians and scientists, until the exact mechanisms are known we must keep an open mind. Thus, even though investigative efforts like that reported by Li and colleagues¹ might be subjected to criticism, we are obligated to consider them.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

The inability to solve this pathogenetic riddle about rheumatic fever represents more than simply the intellectual challenge. Available data confirm this cardiovascular disease to be a continuing problem for medical and public health communities even in industrialising countries.^15,¹⁶ It is very difficult to control any disease process until the pathogenetic mechanisms are understood. The fact that penicillin has clearly failed to eradicate this disease process is irrefutable proof to many of the need for more laboratory, epidemiological, and clinical research. Continuing investigation is imperative to either separate cardiac sequelae of these two vastly different microorganisms (group A streptococci and herpes simplex I) or to determine if the cardiac or valvar damage represents a similar form of end organ damage from two very different infectious agents. The challenge is obvious.

Autoimmunity and RHD

RHD follows ARF in 30-45% of cases. ARF involves all three layers of the heart, as a result of cytokines and other inflammatory molecules which are released to act against the streptococci, as well as attack by immune cells upon the cardiac tissues.

A subspecies of T lymphocytes called CD4+ T cells and macrophages are prominent in this attack, and other molecules such as VCAM-1 help these cells attach to the valves. A genetic susceptibility is necessary for RHD to occur.

The basic mechanism of RHD is called T-cell molecular mimicry. This means that antigens on group A streptococci stimulate the activation of CD4+ T cells which then cross-react with similar peptides in the heart tissue.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

The antigen-T cell reaction of course leads to destruction of the tissue being attacked, which is in this case the heart valve endocardium. Other soluble molecules such as TNF-α, IL-1 and IL-2 are overproduced in ARF by monocytes in peripheral blood. These cytokines promote acute and long-term inflammatory responses which seem to continue as a chronic process even after the infective agent itself has died out.

The lesion, called the Aschoff nodule, is the characteristic feature of an RHD lesion, and consists of a clump of granuloma cells. It passes through different stages and contains a variety of immune cells. Their growth and progression is linked to the production of these cytokines, first by the monocytes in the acute phase and thereafter by T cells as the lesion becomes chronic.

Steps in pathogenesis

The first step is a pharyngeal infection with Streptococcus pyogenes, followed by the presentation of antigens to the immune T and B cells. It is noteworthy that skin infections with the same strain of bacteria do not result in ARF.

Activation of CD4+ cells leads to the production of specific acute and chronic phase antibodies (IgM and IgG, respectively) by B lymphocytes.

These antibodies and activated T cells react with structurally similar proteins or peptides in heart tissue, which is called cross-reaction. As a result, the heart becomes inflamed. Joints develop swelling and pain due to the accumulation of immune complexes, formed by antigen-antibody combinations. Chorea and skin rashes or nodules are other manifestations of this immune activation, in the basal ganglia and the skin respectively.

The molecules which mimic each other may be a spiral protein in M protein and N-acetyl-beta-D-glucosamine antigens found in S. pyogenes, and myosin in the cardiac muscle. In other words, these molecules share some similar antigenic structures with myosin, the human muscle protein. The antibodies formed against these cross-react with tissue in the human heart valve as well.

The overexpression of VCAM-1 molecules causes CD4+ cells to stick to and burrow into the valve endothelium, activating a cellular immune response in the valve. This produces an inflammation of the valve tissue with the growth of new blood vessels.

This leads to further availability of T cells from the increased blood supply brought by these vessels. More and more spots of antigenic attraction may occur on the valve, on other proteins such as vimentin and tropomyosin, so that the T-cell attack extends to more areas. Granulomas (called Aschoff bodies) form underneath the endocardial (innermost) layer of the heart.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Calcification also occurs as part of this inflammation and is linked to the level of a chemical called osteopontin in the blood. Other markers of inflammation such as CRP and oxidation products of proteins are also raised in the blood of patients with RHD.

While this is the accepted mechanism of RHD, newer research is focusing on the endothelium covering the heart valves as the target of immunologic damage in this condition. Hopefully, this will shed light on the paradox of why only throat infection with group A streptococci causes ARF, and not skin infection with the same organism.

Complications include heart failure, which means the heart is unable to pump blood effectively. The strain causes the heart to enlarge. Other complications of RHD include infection of damaged heart valves (infective endocarditis) and stroke due to clots forming in the enlarged heart or on damaged valves. These clots then break off (embolise) and cause blockage in blood vessels in the brain and stroke.

Diagnosis of rheumatic heart disease

Diagnosis may include:

Physical examination – while a heart murmur may suggest RHD, many patients with RHD do not have a murmur
Medical history – including evidence of past ARF or strep infection
Chest x-ray – to check for enlargement of the heart or fluid on the lungs
Electrocardiogram (ECG) – to check if the chambers of the heart have enlarged or if there is an abnormal heart rhythm (arrhythmia)
Echocardiogram – to check the heart valves for any damage or infection and assessing if there is heart failure. This is the most useful test for finding out if RHD is present.

Treatment of rheumatic heart disease

Treatment depends on the severity of rheumatic heart disease, but may include:

Hospital admission to treat heart failure
Antibiotics for infection (especially of the heart valves)
Blood-thinning medicine to prevent stroke or thin blood for replacement valves
Balloons inserted through a vein to open up stuck valves
Heart valve surgery to repair or replace damaged heart valves.

Complications of rheumatic heart disease

Medical treatment of rheumatic heart disease includes reducing the risk of complications. Options may include:

Regular check-ups with a cardiologist (heart specialist) to monitor the heart
Up-to-date flu (influenza and pneumococcal) vaccinations
Regular (preventative) antibiotic to prevent Group A Streptococcus throat infections and recurring ARF
Early presentation, diagnosis and, where appropriate, antibiotic treatment of sore throats
Good dental hygiene (tooth brushing and flossing, dental check-ups, fluoridated water supply), as oral bacteria entering the bloodstream can increase the risk of complications such as inflammation of the inner lining of the heart
Antibiotics – may be given to some people before some dental or surgical procedures to prevent bacterial infection of the damaged areas of the heart
Good prenatal care, as pregnancy can make rheumatic heart disease worse.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Prevention of rheumatic heart disease

RHD is a complication of untreated ARF. People who have had ARF are at increased risk of developing RHD. Prompt diagnosis of ARF and taking preventative antibiotics can prevent RHD. Prophylactic antibiotics are continued until the person is 20 to 40 years old, depending on the time of the last episode of ARF and whether they have RHD or not.

Ideally, ARF and RHD can be prevented. Antibiotic therapy (such as penicillin) to treat GroupA Streptococcus throat infection can dramatically reduce the risk of ARF and its complication, rheumatic heart disease. If ARF or RHD do occur, long-term antibiotics can reduce progression to more severe disease.

Symptoms of RHD

RHD does not always cause symptoms. When it does, symptoms may include:

Chest pain
Heart palpitations
Breathlessness on exertion
Breathing problems when lying down (orthopnoea)
Waking from sleep with the need to sit or stand up (paroxysmal nocturnal dyspnoea)
Swelling (oedema)
Fainting (syncope)
Stroke
Fever associated with infection of damaged heart valves.

Rheumatic fever explained

RHD affects heart valves

The heart is a double pump with four chambers. Each chamber is sealed with a valve. The valves open and close in one direction only, so that the blood cannot flow backwards.

Diagnosis of rheumatic heart disease

Diagnosis may include:

Physical examination – while a heart murmur may suggest RHD, many patients with RHD do not have a murmur
Medical history – including evidence of past ARF or strep infection
Chest x-ray – to check for enlargement of the heart or fluid on the lungs
Electrocardiogram (ECG) – to check if the chambers of the heart have enlarged or if there is an abnormal heart rhythm (arrhythmia)
Echocardiogram – to check the heart valves for any damage or infection and assessing if there is heart failure. This is the most useful test for finding out if RHD is present.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Treatment of rheumatic heart disease

Treatment depends on the severity of rheumatic heart disease, but may include:

Hospital admission to treat heart failure
Antibiotics for infection (especially of the heart valves)
Blood-thinning medicine to prevent stroke or thin blood for replacement valves
Balloons inserted through a vein to open up stuck valves
Heart valve surgery to repair or replace damaged heart valves.

Complications of rheumatic heart disease

Medical treatment of rheumatic heart disease includes reducing the risk of complications. Options may include:

Regular check-ups with a cardiologist (heart specialist) to monitor the heart
Up-to-date flu (influenza and pneumococcal) vaccinations
Regular (preventative) antibiotic to prevent Group A Streptococcus throat infections and recurring ARF
Early presentation, diagnosis and, where appropriate, antibiotic treatment of sore throats
Good dental hygiene (tooth brushing and flossing, dental check-ups, fluoridated water supply), as oral bacteria entering the bloodstream can increase the risk of complications such as inflammation of the inner lining of the heart
Antibiotics – may be given to some people before some dental or surgical procedures to prevent bacterial infection of the damaged areas of the heart
Good prenatal care, as pregnancy can make rheumatic heart disease worse.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Prevention of rheumatic heart disease

Rheumatic heart disease (RHD) is damage to one or more heart valves that remains after an episode of acute rheumatic fever (ARF) is resolved. It is caused by an episode or recurrent episodes of ARF, where the heart has become inflamed. The heart valves can remain stretched and/or scarred, and normal blood flow through damaged valves is interrupted. Blood may flow backward through stretched valves that do not close properly, or may be blocked due to scarred valves not opening properly. When the heart is damaged in this way, the heart valves are unable to function adequately, and heart surgery may be required.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Untreated, RHD causes heart failure and those affected are at risk of arrhythmias, stroke, endocarditis and complications of pregnancy. These conditions cause progressive disability, reduce quality of life and can cause premature death in young adults. Heart surgery can manage some of these problems and prolong life but does not cure RHD.

Although there’s no single test for rheumatic fever, diagnosis is based on medical history, physical exam and certain test results.

Blood tests
If your child was already diagnosed with a strep infection, your doctor might not order additional tests for the bacterium. If your doctor orders a test, it will most likely be a blood test that can detect antibodies to the strep bacterium circulating in the blood. The actual bacterium might no longer be detectable in your child’s throat tissues or blood.

Your doctor also is likely to check for inflammation in your child’s blood by measuring C-reactive protein and the erythrocyte sedimentation rate.

Electrocardiogram (ECG or EKG)
This test — also called an ECG or EKG — records electrical signals as they travel through your child’s heart. Your doctor can look for patterns among these signals that indicate inflammation of the heart or poor heart function.

Echocardiogram
Using sound waves to produce live-action images of the heart, this test can enable your doctor to detect heart abnormalities.

Treatment
The goals of treatment for rheumatic fever are to destroy remaining group A streptococcal bacteria, relieve symptoms, control inflammation and prevent recurrences.

Treatments include:

Antibiotics. Your child’s doctor will prescribe penicillin or another antibiotic to eliminate remaining strep bacteria.

After your child has completed the full antibiotic treatment, your doctor will begin another course of antibiotics to prevent recurrence of rheumatic fever. Preventive treatment will likely continue through age 21 or until your child completes a minimum five-year course of treatment, whichever is longer.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

People who have had heart inflammation during rheumatic fever might be advised to take the preventive antibiotic treatment for 10 years or longer.

Anti-inflammatory treatment. Your doctor will prescribe a pain reliever, such as aspirin or naproxen (Naprosyn), to reduce inflammation, fever and pain. If symptoms are severe or your child isn’t responding to the anti-inflammatory drugs, your doctor might prescribe a corticosteroid.
Anticonvulsant medications. For severe involuntary movements caused by Sydenham chorea, your doctor might prescribe an anticonvulsant, such as valproic acid (Depakene) or carbamazepine (Carbatrol, Tegretol, others).
Long-term care
Discuss with your doctor what type of follow-up and long-term care your child will need. Heart damage from rheumatic fever might not show up for years. When your child grows up, he or she needs to include the information in his or her medical history and get regular heart exams.

Acute rheumatic fever (ARF) is an illness that may occur after a bacterial infection with group A Streptococcus bacteria. It is most common in children aged 5 to 14 years. Rheumatic heart disease (RHD) occurs as a complication of ARF. Although ARF and RHD are rare in the general Australian population, they are a common cause of heart disease in Aboriginal and Torres Strait Islander children and also occur among some migrant populations (particularly from Africa, Asia, the Middle East and Pacific Islands).

ARF is caused by an autoimmune reaction to the bacterial infection. This means that the immune system mistakenly attacks healthy body tissue. In some people, the body’s immune response to a group A streptococcal infection results in inflammation of the heart, joints, skin and brain (ARF). Only a small percentage of people with streptococcal infection develop ARF, but those who have had ARF before are more likely to have repeated episodes.

Group A Streptococcus

Infection with group A Streptococcus may cause sore throat, tonsillitis or skin infection. Streptococci are spread when an infected person talks, coughs or sneezes small droplets containing infectious agents into the air. The droplets in the air may be breathed in by those nearby. The droplets may contaminate hands or objects such as drinking cups or eating utensils. Sometimes spread occurs by eating contaminated food. Sometimes spread occurs by direct contact with infected wounds or skin sores.

There is a period of about 3 weeks between the initial infection with group A Streptococcusand ARF symptoms.

Symptoms

Symptoms of ARF include:

fever
malaise (feeling of being unwell)
painful and/or swollen joints (one or many joints) – usually affecting the elbows, wrists, hips, knees and ankles
chest pain, difficulty breathing, or a rapid heartbeat and heart murmur that may be detected by doctors
jerky movements and difficulty walking.

Rarely ARF may present with:

painless lumps under the skin, especially at the elbows, wrists, knees, ankles and spine
skin rash on the trunk, arms and legs.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

What is RHD?

Inflammation of the heart that occurs with ARF can result in permanent damage to the heart, particularly the heart valves. This is called rheumatic heart disease (RHD). The likelihood of developing RHD increases with repeated episodes of ARF.

Healthy heart valves open and shut with each heartbeat, sealing each of the four chambers of the heart and preventing blood flowing in the wrong direction. With RHD the heart valves are unable to open and shut properly. This prevents the heart from pumping blood efficiently and puts excessive strain on the heart. People with RHD may have no symptoms for years or only mild symptoms (such as shortness of breath with exercise or on lying down, tiredness, weakness or palpitations) until complications develop.

Complications

Longer term complications of RHD are severe and include:

heart failure (when the heart cannot pump enough to meet the needs of the body)
infection of damaged heart valves
stroke due to clots forming in the heart or on damaged valves that break off and block blood vessels in the brain
rapid heartbeat or other disturbed heart rhythms.

Diagnosis of ARF and RHD

There is no single specific test to diagnose ARF. Diagnosis of ARF is based upon a medical assessment along with various tests such as echocardiography (ultrasound scan of the heart) to check the valves, an ECG (electrocardiogram) which shows the rhythm of the heart, blood tests and throat swabs (to look for recent group A streptococcal infection).

RHD is also diagnosed by a medical assessment and echocardiography.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Treatment

People with ARF are usually very ill and require hospital admission. Treatment includes antibiotics (usually penicillin), and other medicines for fever, arthritis and heart failure. Heart surgery may be needed if there is life threatening damage to the heart valves.

Once someone is diagnosed with ARF or RHD he or she requires long term treatment with penicillin injections every 3 to 4 weeks. The length of treatment is at least 10 years, and may be longer depending on the severity of damage to the heart valves. Penicillin injections are given to prevent further episodes of ARF. This in turn prevents further damage to the heart valves.

Regular checks with the general practitioner and specialists such as a cardiologist or paediatrician are required to monitor the condition of the heart. Regular visits to the dentist are also important to prevent any infections in the mouth from spreading and damaging the heart valves.

Prevention

Get sore throats and skin sores checked at a health clinic especially if you have a history of ARF or if you are in a high risk group (Aboriginal and Torres Strait Islander people and migrants from countries where ARF and RHD are common).
Ensure all cuts and skin sores are kept clean.
Maintain good personal hygiene – brush teeth at least twice a day and have a bath or shower daily.
Always follow good hand washing procedures.

SA Rheumatic Heart Disease Register

In South Australia, a consent-based RHD Register is operating and cases of ARF and RHD should be reported to the Register. The Register supports people with ARF and RHD and their health professionals in providing appropriate ongoing care.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

1 – In South Australia the law requires doctors and laboratories to report some infections or diseases to SA Health. These infections or diseases are commonly referred to as ‘notifiable conditions’.

Not everyone who has a streptococcal infection will develop rheumatic fever (RF), but if RF does develop, symptoms normally appear 2 to 4 weeks after infection.

It most commonly affects boys and girls aged 5 to 15 years, but it can occur in adults and younger children. Neurologic complications seem to be more common in females.

RF can have long-term complications, the most common being rheumatic heart disease (RHD) which develops in 30 to 45 percent of those with RF. Worldwide, RHD is responsible for 230,000 – 500,000 deaths per year.

Before the widespread introduction of antibiotics, RF was a leading cause of acquired heart disease in developed nations, but it is now relatively rare in these countries. Thanks to routine treatment of Strep throat, RF now only occurs in about 0.04-0.06 cases per every 1,000 children in mainland U.S.RHD is by far the most important form of acquired heart disease in children and young adults living in developing countries (which are inhabited by 80 percent of the world’s population); RHD accounts for approximately 15 percent of all patients with heart failure (HF) in endemic countries [2,3].Medical therapy in rheumatic heart disease includes attempts to prevent rheumatic fever (and thus rheumatic heart disease). In patients who develop rheumatic heart disease, therapy is directed toward eliminating the group A streptococcal pharyngitis (if still present), suppressing inflammation from the autoimmune response, and providing supportive treatment for congestive heart failure. Following the resolution of the acute episode, subsequent therapy is directed towards preventing recurrent rheumatic heart disease in children and monitoring for the complications and sequelae of chronic rheumatic heart disease in adults.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Although having rheumatic fever leaves a child more susceptible to heart damage, it does not always permanently damage the heart. However, when the inflammation caused by rheumatic fever leaves one or more of the heart valves scarred, the result is rheumatic heart disease. The mitral valve and the aortic valve are usually the ones damaged by the disease. Years later, the mitral valve may become narrowed, a condition known as mitral stenosis.

Treatment of acute rheumatic fever includes antibiotics to treat the strep infection and additional medications to ease the inflammation of the heart and other symptoms. Usually aspirin is given in large doses until the joint inflammation goes away; rarely, steroids are needed. Once the acute illness has gone away, patients need to take penicillin, or an equivalent antibiotic, for many years to prevent recurrences. This is a very important treatment because the risk of heart valve damage increases if rheumatic fever recurs.

Most often the valve leak caused by the disease is mild and does not need treatment. If the leak is severe enough to strain and enlarge the heart, surgery may be needed to eliminate the leak. This surgery may involve repair of the damaged valve. Sometimes the valve is too badly damaged to repair, in which case it must be replaced by an artificial valve.

Rheumatic heart disease is a condition where the heart valves have been permanently damaged by rheumatic fever. The heart valve damage may start shortly after untreated or under-treated streptococcal infection such as strep throat or scarlet fever. An immune response causes an inflammatory condition in the body. This can result in ongoing valve damage.

What causes rheumatic heart disease?
Rheumatic heart disease is caused by rheumatic fever. This is an inflammatory disease that can affect many connective tissues, especially in the heart, joints, skin, or brain. The heart valves can be inflamed and become scarred over time. This can result in narrowing or leaking of the heart valve. This makes it harder for the heart to work normally. This may take years to develop and can lead to heart failure. Rheumatic fever can occur at any age. But it often occurs in children ages 5 to 15. It’s rare in developed countries like the U.S.

Who is at risk for rheumatic heart disease?
Untreated or under-treated strep infections can increase the risk for rheumatic heart disease. Children who get repeated strep throat infections are at the most risk for rheumatic fever and rheumatic heart disease.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

What are the symptoms of rheumatic heart disease?
A recent history of strep infection or rheumatic fever is key to the diagnosis of rheumatic heart disease. Symptoms of rheumatic fever vary. They typically start 1 to 6 weeks after a bout of strep throat. In some cases, the infection may have been too mild to be recognized. Or it may be gone by the time the person sees a healthcare provider.

These are the most common symptoms of rheumatic fever:

Fever

Swollen, tender, red and very painful joints (very often the knees and ankles)

Lumps under the skin (nodules)

Red, raised, lattice-like rash, often on the chest, back, and belly

Shortness of breath and chest discomfort

Uncontrolled movements of arms, legs, or facial muscles

Weakness

Symptoms of rheumatic heart disease depend on the degree of valve damage and may include:

Shortness of breath (very often with activity or when lying down)

Chest pain

Swelling

How is rheumatic heart disease diagnosed?
People with rheumatic heart disease will have or recently had a strep infection. A throat culture or blood test may be used to check for strep.

They may have a murmur or rub that may be heard during a routine physical exam. The murmur is caused by the blood leaking around the damages valve. The rub is caused when the inflamed heart tissues move or rub against each other.

Along with a complete health history and physical exam, tests used to diagnose rheumatic heart disease may include:

Echocardiogram (echo). This test uses sound waves to check the heart’s chambers and valves. The echo sound waves create a picture on a screen as a handheld ultrasound probe (transducer) is passed over the skin over the heart. Echo can show damage to the valve flaps, backflow of blood through a leaky valve, fluid around the heart, and heart enlargement. It’s the most useful test for diagnosing heart valve problems. For more in-depth pictures you may be given sedation and the probe is put into the throat (transesophageal echo or TEE).

Electrocardiogram (ECG). This test records the strength and timing of the heart’s electrical activity. It shows abnormal rhythms (arrhythmias or dysrhythmias). And it can sometimes find heart muscle damage. Small sensors are taped to your skin to pick up the electrical activity.

Chest X-ray. An X-ray may be done to check your lungs and see if your heart is enlarged.

Cardiac MRI. This is an imaging test that takes detailed pictures of the heart. It may be used to get a more exact look at the heart valves and heart muscle.

Blood tests. Certain blood tests may be used to look for infection and inflammation.

A study of RHD cases estimated that in 2015, there were globally 33.4 million cases of RHD, 10.5 million disability-adjusted life-years due to RHD, and 319,400 deaths due to RHD [4]. The global mortality burden of RHD decreased by nearly 50 percent from 1990 to 2015, but the prevalence varied widely among countries and was highest in Oceania, central sub-Saharan Africa, and South Asia. Estimated age-standardized prevalence of RHD in 2015 was 3.4 cases per 100,000 population in nonendemic countries and 444 cases per 100,000 population in endemic countries. Twenty countries with an endemic pattern of RHD had an age-standardized prevalence exceeding 1 percent [4].

RHD is a disease affecting predominantly those living in poverty with inadequate access to health care and unchecked exposure to group A streptococcus [5]. The impact of socioeconomic status is illustrated by a study from Kinshasa where the prevalence based on clinical examination was 22.2 per 1000 among children who lived in slums but only 4 per 1000 among children attending the city schools [6]. A later report outlined the increased risk of RHD in association with overcrowding and unemployment as well as overcrowding and distance from the nearest health center [7]. The importance of socioeconomic factors is further underscored by the virtual disappearance of RHD in industrialized countries since the mid-20^th century, which started well before the introduction of penicillin. By contrast, RHD is still endemic in Africa, Asia, South America, and developing communities of Australasia [4,8-12].

Rheumatic fever is an inflammatory disease that may affect many connective tissues of the body, especially those of the heart, joints, brain or skin. It usually starts out as a strep throat (streptococcal) infection. Anyone can get acute rheumatic fever, but it usually occurs in children between the ages of 5 and 15 years. About 60% of people with rheumatic fever develop some degree of subsequent heart disease.

There is currently no single test to diagnose acute rheumatic fever (ARF). Diagnosis is a doctor’s decision based on clinical assessment and the identification of a number of signs and symptoms that are associated with the illness.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Potential signs and symptoms of ARF are divided into two categories: major and minor. Major signs and symptoms are strongly associated with ARF and include carditis (swelling of the heart), arthritis (pain, redness and swelling of one or more joints), Sydenham’s chorea (strange movements of the body and face), erythema marginatum (painless skin pigmentation), and subcutaneous nodules (small lumps under the skin). Minor signs and symptoms are used to help support the diagnosis. These include fever, arthralgia (generalised joint aches), blood tests that suggest general illness, and changes seen on heart electrocardiogram.

Every part of the heart, including the outer sac (the pericardium), the inner lining (the endocardium) and the valves may be damaged by inflammation caused by acute rheumatic fever. However, the most common form of rheumatic heart disease affects the heart valves, particularly the mitral valve. It may take several years after an episode of rheumatic fever for valve damage to develop or symptoms to appear.

Antibiotics can prevent streptococcal infection from developing into rheumatic fever. Any child with a persistent sore throat should have a throat culture to check for strep infection. Penicillin or another antibiotic will usually prevent strep throat from developing into rheumatic fever.

Symptoms

Symptoms of heart valve problems, which are often the result of rheumatic heart disease, can include: chest pain, excessive fatigue, heart palpitations (when the heart flutters or misses beats), a thumping sensation in the chest, shortness of breath, and swollen ankles, wrists or stomach.

Treatment

If heart damage from rheumatic fever is identified in childhood or young adulthood, daily antibiotics may be required until the age of 25 or 30, to help prevent recurrence of rheumatic fever and avoid the development of infective bacterial endocarditis, an infection of the heart valves or lining of the heart. Additional treatment will depend on the type of heart damage.

Symptoms

Rheumatic fever is a complication of some types of streptococcal infection.

RF is caused by a reaction to the bacteria that cause strep throat, so that diagnosis and treatment of this condition can prevent it from developing into RF.

Symptoms of strep throat include:

sore throat
headache
swollen, tender lymph nodes
trouble swallowing
nausea and vomiting
red skin rash
high temperature
swollen tonsils
abdominal pain

Signs and symptoms generally develop 2 to 4 weeks after a streptococcal infection.

Some individuals will experience just one or two of the following symptoms, but others may experience most of them:

fatigue
rapid heart rate
decreased ability to exercise
joint pain and swelling
fever
splotchy rash
uncontrollable twitching and movements

Arthritis, or pain and swelling in the joints, affects 75 percent of patients. It normally starts in the larger joints, such as the knees, ankles, wrists, and elbows, before moving to other joints. This inflammation normally resolves within 4-6 weeks, without causing permanent damage.

Inflammation of the heart can lead to chest pain, palpitations, a sensation that the heart is fluttering or pounding hard, panting, and shortness of breath, and fatigue.

While rheumatic heart disease (RHD) may develop after a single bout of acute rheumatic fever (ARF), it is typically associated with recurrent episodes of ARF. ARF usually occurs during childhood between the ages of 5 and 15 years. RHD can damage any part of the heart including the valves, the lining of the heart or the heart muscle, but more often damages the heart valves, especially those on the left side of the heart.

Rheumatic heart disease (RHD) is common in remote Aboriginal communities

Symptoms of RHD

RHD does not always cause symptoms. When it does, symptoms may include:

Chest pain
Heart palpitations
Breathlessness on exertion
Breathing problems when lying down (orthopnoea)
Waking from sleep with the need to sit or stand up (paroxysmal nocturnal dyspnoea)
Swelling (oedema)
Fainting (syncope)
Stroke
Fever associated with infection of damaged heart valves.

Acute rheumatic fever can be undiagnosed and this can cause failure to prevent or recognise rheumatic heart disease. Failure to recognise ARF and limited access to healthcare can contribute to the under-diagnosis of ARF. Caring for Hospitalized Adolescents with Heart Murmur Essay Paper
The heart is a double pump with four chambers. Each chamber is sealed with a valve. The valves open and close in one direction only, so that the blood cannot flow backwards.

Diagnosis of rheumatic heart disease

Diagnosis may include:

Physical examination – while a heart murmur may suggest RHD, many patients with RHD do not have a murmur
Medical history – including evidence of past ARF or strep infection
Chest x-ray – to check for enlargement of the heart or fluid on the lungs
Electrocardiogram (ECG) – to check if the chambers of the heart have enlarged or if there is an abnormal heart rhythm (arrhythmia)
Echocardiogram – to check the heart valves for any damage or infection and assessing if there is heart failure. This is the most useful test for finding out if RHD is present.

Treatment of rheumatic heart disease

Treatment depends on the severity of rheumatic heart disease, but may include:

Hospital admission to treat heart failure
Antibiotics for infection (especially of the heart valves)
Blood-thinning medicine to prevent stroke or thin blood for replacement valves
Balloons inserted through a vein to open up stuck valves
Heart valve surgery to repair or replace damaged heart valves.

Complications of rheumatic heart disease

Medical treatment of rheumatic heart disease includes reducing the risk of complications. Options may include:

Regular check-ups with a cardiologist (heart specialist) to monitor the heart
Up-to-date flu (influenza and pneumococcal) vaccinations
Regular (preventative) antibiotic to prevent Group A Streptococcus throat infections and recurring ARF
Early presentation, diagnosis and, where appropriate, antibiotic treatment of sore throats
Good dental hygiene (tooth brushing and flossing, dental check-ups, fluoridated water supply), as oral bacteria entering the bloodstream can increase the risk of complications such as inflammation of the inner lining of the heart
Antibiotics – may be given to some people before some dental or surgical procedures to prevent bacterial infection of the damaged areas of the heart
Good prenatal care, as pregnancy can make rheumatic heart disease worse.

On average, around 50 percent of patients develop carditis or valvulitis, a potentially fatal inflammation of the heart that can have serious, long-term effects. Younger children are more susceptible.

Inflammation of the nerves can lead to symptoms of Sydenham’s chorea, including:Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

chorea, the uncontrollable jerking of knees, elbows, wrists, and ankles
inappropriate crying or laughing
irritability and moodiness
difficulty controlling fine hand movements
problems with balance

Symptoms usually pass within a few months but can last up to 2 years. They are not normally permanent.

Other symptoms include a red, blotchy, skin rash, which appears in 1 in 10 cases. Less common are nosebleeds, abdominal pain, bumps and lumps, or nodules, under the skin, and a high fever over 102 degrees Fahrenheit.

The inflammation may also lead to headache, sweating, vomiting, and weight loss.

Treatment aims to destroy the bacteria, relieve symptoms, control inflammation, and prevent recurrences of RF.

Antibiotics, such as penicillin, may be given to destroy any remaining strep bacteria in the body. Further antibiotics may be prescribed, to prevent recurrence. This may continue for 5-10 years depending on the age of the person and whether or not the heart is affected.

Long-term, and even lifelong, preventive antibiotics may be necessary to prevent recurring inflammation of the heart.

It is important to remove all traces of streptococcal bacteria, as any remaining bacteria can lead to repeated occurrences of RF and a significantly higher risk of heart damage, which can become permanent.

Anti-inflammatory drugs: Naproxen, for example, may help to reduce pain, inflammation, and fever.

Corticosteroids: Prednisone may be given if the patient does not respond to first-line anti-inflammatory medications, or if there is inflammation of the heart.

Aspirin: This is not usually recommended for children aged under 16 years because of the risk of developing Reye’s syndrome, which can cause liver and brain damage, and even death, but an exception is usually made in cases of RA because the benefits are greater than the risks.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Anticonvulsant medications: These can treat severe chorea symptoms. Examples include valproic acid (Depakene or Stavzor), carbamazepine (Carbatrol or Equetro), haloperidol (Haldol) and risperidone (Risperdal).

Anyone who has RF as a child will need to inform their doctor as they get older because heart damage can appear many years later.

Diagnosis

The doctor will ask about the patient’s symptoms and recent medical history. They will pay particular attention to any recent illness along with the following:

swelling, pain, and stiffness in the joints
any jerky, involuntary movements
a red or pink skin rash
small nodules or lumps and bumps under the skin, especially on the elbows, ankles, knees, and knuckles
irregular heart rhythm

Tests may include:

Electrocardiogram (EKG): An electrical tracing of the heart to detect abnormal heart rhythms suggesting inflammation
Echocardiography: An ultrasound of the heart to look for inflammation or heart valve damage
Blood tests

Additional tests can detect specific strep infections.

Complications

Rheumatic fever can cause heart disease in children.

Symptoms of RF, and specifically inflammation, may persist for weeks, months, or longer, causing long-term problems.

Rheumatic heart disease (RHD) is the most common and most serious complication.

Globally, RHD is estimated to affect more than 15 million people per year and cause more than 230,000 deaths.

The inflammation causes permanent damage to the heart, most commonly the mitral valve, the valve between the upper and lower chambers of the left side of the heart.

This can lead to:

Valvular stenosis: The valve narrows, causing a drop in blood flow
Valvular regurgitation: Blood flows in the wrong direction because of a leak
Heart muscle damage: Inflammation weakens the heart muscle so that the heart cannot pump properly

Other conditions that may develop if there is damage to heart tissue, the mitral valve, or other heart valves include:

Heart failure: This is a serious condition in which the heart is not pumping blood throughout the body efficiently. This can affect the left side, the right side, or both sides of the heart.
Atrial fibrillation: An abnormal heart rhythm where the upper chambers of the heart (the atria) do not coordinate with the lower part of the heart (the ventricles). This causes the heart muscle to contract irregularly, excessively fast, or both making its pumping ability inefficient. This abnormal rhythm can also lead to a stroke.

RF is now rare in developed countries, but it remains a risk elsewhere. Researchers continue to seek effective ways to prevent RF and its complications.

Causes

The main cause of RF is Group A streptococcus (GAS), a bacteria that can cause infections such as Strep throat with or without scarlet feverand skin infections like impetigo, and cellulitis.

However, not all strains of streptococcal bacteria will lead to RF, and not everyone who has a GAS infection will develop RF.

Genetic factors may increase the risk. The chance of having RF appears to be higher if another family member has had it.

The exact link between group A strep infection and RF remains unclear, but scientists believe that it is not the bacteria itself that causes the disease, but rather the immune system’s faulty reaction to it.

Strep bacteria have a protein that resembles one found in some body tissues. Immune system cells that would usually target the bacteria may start attacking the body’s own tissues instead, as if they are toxins or infectious agents.

In RF, the tissues that they attack are those of the heart, joints, central nervous system (CNS) and skin. These tissues react by becoming inflamed.

If a patient with Strep bacteria takes a complete course of antibiotic treatment, the chances of RF developing are very low.

A chest x-ray should be completed to evaluate for cardiomegaly or pulmonary vascular congestion, which can be signs of congestive heart failure.

Though there are many different types of congenital heart defects, they can be divided into three main categories:

In heart valve defects, the valves inside the heart that direct blood flow may close up or leak. This interferes with the heart’s ability to pump blood correctly.
In heart wall defects, the natural walls that exist between the left and right sides and the upper and lower chambers of the heart may not develop correctly, causing blood to back up into the heart or to build up in places where it doesn’t belong. The defect puts pressure on the heart to work harder, which may result in high blood pressure.
In blood vessel defects, the arteries and veins that carry blood to the heart and back out to the body may not function correctly. This can reduce or block blood flow, leading to various health complications.

Cyanotic and Acyanotic Congenital Heart Disease

Many doctors classify congenital heart disease as either cyanotic congenital heart disease or acyanotic congenital heart disease. In both types, the heart isn’t pumping blood as efficiently as it should. The main difference is that cyanotic congenital heart disease causes low levels of oxygen in the blood, and acyanotic congenital heart disease doesn’t. Babies with reduced oxygen levels may experience breathlessness and a bluish tint to their skin. Babies who have enough oxygen in their blood don’t display these symptoms, but they may still develop complications later in life, such as high blood pressure.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

What Are the Symptoms of Congenital Heart Disease?

A congenital heart defect is often detected during a pregnancy ultrasound. If your doctor hears an abnormal heartbeat, for instance, they may further investigate the issue by performing certain tests. These may include an echocardiogram, a chest X-ray, or an MRI scan. If a diagnosis is made, your doctor will make sure the appropriate specialists are available during delivery.

In some cases, the symptoms of a congenital heart defect may not appear until shortly after birth. Newborns with heart defects may experience:

bluish lips, skin, fingers, and toes
breathlessness or trouble breathing
feeding difficulties
low birth weight
chest pain
delayed growth

In other cases, the symptoms of a congenital heart defect may not appear until many years after birth. Once symptoms do develop, they may include:

abnormal heart rhythms
dizziness
trouble breathing
fainting
swelling
fatigue

What Causes Congenital Heart Disease?

Congenital heart disease occurs as a result of an early developmental problem in the heart’s structure. The defect typically interferes with the normal flow of blood through the heart, which may affect breathing. Although researchers aren’t exactly sure why the heart fails to develop correctly, suspected causes include the following:

The heart defect may run in families.
Taking certain prescription drugs during pregnancy puts a child at a higher risk for a heart defect.
Using alcohol or illegal drugs during pregnancy can increase a child’s risk of having a heart defect.
Mothers who had a viral infection during the first trimester of pregnancy are more likely to give birth to a child with a heart defect.
Increased blood sugar levels, such as occurs with diabetes, may affect childhood development.

How Is Congenital Heart Disease Treated?

The treatment for a congenital heart defect depends on the type and severity of the defect. Some babies have mild heart defects that heal on their own with time. Others may have severe defects that require extensive treatment. In these cases, treatment may include the following:

Medications

There are various medications that can help the heart work more efficiently. Some can also be used to prevent blood clots from forming or to control an irregular heartbeat.

Implantable Heart Devices

Some of the complications associated with congenital heart defects can be prevented with the use of certain devices, including pacemakers and implantable cardioverter defibrillators (ICDs). A pacemaker can help regulate an abnormal heart rate, and an ICD may correct life-threatening irregular heartbeats.

Catheter Procedures

Catheterization techniques allow doctors to repair certain congenital heart defects without surgically opening the chest and heart. During these procedures, the doctor will insert a thin tube into a vein in the leg and guide it up to the heart. Once the catheter is in the correct position, the doctor will use small tools threaded through the catheter to correct the defect.

Open-Heart Surgery

This type of surgery may be needed if catheter procedures aren’t enough to repair a congenital heart defect. A surgeon may perform open-heart surgery to close holes in the heart, repair heart valves, or widen blood vessels.

Heart Transplant

In the rare cases in which a congenital heart defect is too complex to fix, a heart transplant may be needed. During this procedure, the child’s heart is replaced with a healthy heart from a donor.

Congenital Heart Disease in Adults

Depending on the defect, diagnosis and treatment may begin shortly after birth, during childhood, or in adulthood. Some defects don’t cause any symptoms until the child becomes an adult, so diagnosis and treatment may be delayed. In these cases, the symptoms of a newly discovered congenital heart defect may include:

shortness of breath
chest pain
a reduced ability to exercise
being easily fatigued

The treatment for congenital heart disease in adults can also vary depending on the severity of the heart defect. Some people may only need to monitor their condition closely, and others may require medications and surgeries.

In some cases, defects that may have been treated in childhood can present problems again in adulthood. The original repair may no longer be effective or the initial defect may have become worse over time. Scar tissue that developed around the original repair may also end up causing problems, such as heart arrhythmias.

Regardless of your situation, it’s important to continue seeing your doctor for follow-up care. Treatment may not cure your condition, but it can help you maintain an active, productive life. It will also reduce your risk for serious complications, such as heart infections, heart failure, and stroke.

A transthoracic echocardiogram is more sensitive and specific than auscultation during physical examination for detection of rheumatic heart disease. Rheumatic heart disease seen on transthoracic echo without evidence of a murmur on auscultation is “subclinical rheumatic heart disease.”[8]

Mitral regurgitation is the most common presentation of rheumatic heart disease in young people. However, rheumatic heart disease is the most common cause of mitral stenosis worldwide.[9]Common descriptions of the mitral valve on echocardiography are ‘dog-leg’ ‘elbow’ or ‘hockey-stick’ deformities, which all help describe the thickening and restricted motion of the anterior mitral valve leaflet Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Rheumatic heart disease, often neglected by media and policy makers, is a major burden in developing countries where it causes most of the cardiovascular morbidity and mortality in young people, leading to about 250,000 deaths per year worldwide. The disease results from an abnormal autoimmune response to a group A streptococcal infection in a genetically susceptible host. Acute rheumatic fever–the precursor to rheumatic heart disease–can affect different organs and lead to irreversible valve damage and heart failure. Although penicillin is effective in the prevention of the disease, treatment of advanced stages uses up a vast amount of resources, which makes disease management especially challenging in emerging nations. Guidelines have therefore emphasised antibiotic prophylaxis against recurrent episodes of acute rheumatic fever, which seems feasible and cost effective. Early detection and targeted treatment might be possible if populations at risk for rheumatic heart disease in endemic areas are screened. In this setting, active surveillance with echocardiography-based screening might become very important.

Acute rheumatic fever (ARF) is the result of an autoimmune response to pharyngitis caused by infection with group A Streptococcus. The long-term damage to cardiac valves caused by ARF, which can result from a single severe episode or from multiple recurrent episodes of the illness, is known as rheumatic heart disease (RHD) and is a notable cause of morbidity and mortality in resource-poor settings around the world. Although our understanding of disease pathogenesis has advanced in recent years, this has not led to dramatic improvements in diagnostic approaches, which are still reliant on clinical features using the Jones Criteria, or treatment practices. Indeed, penicillin has been the mainstay of treatment for decades and there is no other treatment that has been proven to alter the likelihood or the severity of RHD after an episode of ARF. Recent advances — including the use of echocardiographic diagnosis in those with ARF and in screening for early detection of RHD, progress in developing group A streptococcal vaccines and an increased focus on the lived experience of those with RHD and the need to improve quality of life — give cause for optimism that progress will be made in coming years against this neglected disease that affects populations around the world, but is a particular issue for those living in poverty.

A congenital heart defect (CHD) is a structural problem with the heart that’s present at birth. Such defects result when a mishap occurs during heart development soon after conception – often before the mother is aware that she is pregnant.

Such problems may or may not have a disruptive effect on a person’s circulatory system. But having a congenital heart defect can increase your risk of developing certain medical conditions.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Understanding how defects develop

To understand congenital heart defects, it’s helpful to remember how the heart is meant to function.

A normal heart has valves, arteries and chambers that circulate blood in a recurring pattern: body to heart, heart to lungs, lungs to heart and then heart out to the body. When all the chambers and valves work correctly, blood is pumped through the heart, to the lungs for oxygen, back to the heart and then throughout the body to deliver that oxygen. When valves, chambers, arteries and veins are malformed, this circulation pattern can be impaired.

Congenital heart defects range in severity from simple problems, such as “holes” between chambers of the heart, to very severe malformations, such as the complete absence of one or more chambers or valves.

Associated conditions

A congenital heart defect can increase your risk for certain medical conditions, including:

Pulmonary hypertension
Arrhythmias
Infective endocarditis
Anticoagulation
Congestive heart failure

Congenital heart defects: FAQ

Are all heart problems in children congenital?

No, but most are. There are three general categories of possible childhood heart problems: structural defects, acquired damage and heart rhythm disturbances. These defects are usually, but not always, diagnosed early in life.

Children also can be born with or develop heart rate problems such as slow, fast or irregular heartbeats, known as “arrhythmias.”

Rarely, childhood heart problems are not present at birth. Instead, heart damage may occur during childhood due to infection. This type of heart disease is called “acquired.” Examples of such acquired problems include Kawasaki disease and rheumatic fever (PDF)(link opens in new window).

Who is at risk to have a child with a congenital heart defect?

Anyone can have a child with a congenital heart defect. Out of 1,000 births, at least eight babies will have some form of congenital heart disorder, most of which are mild. If you or other family members have already had a baby with a heart defect, your risk of having a baby with a heart defect may be higher.

Why do congenital heart defects occur?

Most of the time, the cause isn’t known. Although the reason defects occur is presumed to be genetic, only a few genes have been discovered that have been linked to heart defects.

Rarely, the ingestion of some drugs and the occurrence of some infections during pregnancy can cause defects.

How can I tell if my baby or child has a congenital heart defect?

Severe heart disorders generally become evident during the first few months after birth. Some babies are blue or have very low blood pressure shortly after birth. Other defects cause breathing difficulties, feeding problems or poor weight gain.

Minor defects are most often diagnosed during a routine medical checkup. Minor defects rarely cause symptoms. While most heart murmurs in children are normal, some may be due to defects.

How well can people with congenital heart defects function?

Virtually all children with simple defects survive into adulthood. Although exercise capacity may be limited, most people lead normal or nearly normal lives.

With more complex problems, limitations are common. Some children with congenital heart defects have developmental delay or other learning difficulties.

What is the social/financial impact of congenital heart defects?

Successful treatment requires highly specialized care. Treatment for severe congenital heart defects requires extensive financial resources, including the costs associated with hospitalization.

Children with developmental delays also require community- and school-based resources to achieve their full potential.

What is the impact of congenital heart disease on families?

A serious congenital heart defect can put an enormous emotional and financial strain on young families at a vulnerable time.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Patient and family education is an important part of successful coping. Turn to our Support Network, where you can share concerns and gain insights within a community of care.

Chest tube. A drainage tube will be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.

Heart monitor. A machine that constantly displays a picture of your child’s heart rhythm, and monitors heart rate, arterial blood pressure, and other values.

Your child may need other equipment not mentioned here to provide support while in the ICU, or afterward. The hospital staff will explain all of the necessary equipment to you.

Your child will be kept as comfortable as possible with several different medications; some of which relieve pain, and some of which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.

After discharge from the ICU, your child will recuperate on another hospital unit for a few days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while, and these will be explained to you. The staff will give you instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.

Long-term outlook after coarctation of the aorta surgical repair
Most children who have had a coarctation of the aorta surgical repair will live healthy lives. Activity levels, appetite, and growth should eventually return to normal.

Your child’s cardiologist may recommend that antibiotics before major surgeries or procedures, such as dental cleaning, to prevent infection.

As the child grows, the aorta may once again become narrow on occasion. If this happens, a balloon procedure or operation may be necessary to repair the coarctation. Evaluation with MRI is generally recommended. If an aortic aneurysm or dissection is suspected, computed tomography (CT scan) may also be done to further evaluate the anatomy before deciding on treatment options.

Blood pressure management is very important. Often, the blood pressure in the child is elevated after aortic coarctation repair. In that case, medications may be prescribed to help lower the child’s blood pressure.

Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout life.

Consult your child’s health care provider regarding the specific outlook for your chilD

Rheumatic fever (RF) and rheumatic heart disease (RHD) continue to be a major health hazard in most developing countries as well as sporadically in developed economies. Despite reservations about the utility, echocardiographic and Doppler (E&D) studies have identified a massive burden of RHD suggesting the inadequacy of the Jones’ criteria updated by the American Heart Association in 1992. Subclinical carditis has been recognized by E&D in patients with acute RF without clinical carditis as well as by follow up of RHD patients presenting as isolated chorea or those without clinical evidence of carditis. Over the years, the medical management of RF has not changed. Paediatric and juvenile mitral stenosis (MS), upto the age of 12 and 20 yr respectively, severe enough to require operative treatement was documented. These negate the belief that patients of RHD become symptomatic ≥20 years after RF as well as the fact that congestive cardiac failure in childhood indicates active carditis and RF. Non-surgical balloon mitral valvotomy for MS has been initiated. Mitral and/or aortic valve replacement during active RF in patients not responding to medical treatment has been found to be life saving as well as confirming that congestive heart failure in acute RF is due to an acute haemodynamic overload. Pathogenesis as well as susceptibility to RF continue to be elusive. Prevention of RF morbidity depends on secondary prophylaxis which cannot reduce the burden of diseases. Primary prophylaxis is not feasible in the absence of a suitable vaccine. Attempts to design an antistreptococcal vaccine utilizing the M-protein has not succeeded in the last 40 years. Besides pathogenesis many other questions remain unanswered.

ARF incidence rates have been reported to be as high as 155 per 100,000 children aged 5 to 14 years in indigenous populations in North Queensland, Australia (Gray, Brown, & Thomson, 2012) with rates in the Northern Territory reported at 380.1 per 100,000 children in 2002 (Parnaby & Carapetis, 2010). In New Zealand, ARF affects mainly children and teens aged 4–19 years who are predominantly of Māori and Pasifika descent and are living in low socioeconomic regions of the North Island (Jack, et al., 2015). Between 1993 and 2009, the average incidence rates for ARF based on hospitalization data for children 5–14 years were 81.2 per 100,000 for Pasifika children, as compared with 40.2 per 100,000 for Māori children and 2.1 per 100,000 for non-Māori, non-Pasifika children (Milne, Lennon, Stewart, Vander Hoorn, & Scuffham, 2012a).Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

The heart is a muscular pump about the size of a clenched fist. An enlarged heart isn’t a condition in itself, but a symptom of an underlying problem that is causing the heart to work harder than normal.

Older people are at increased risk of having an enlarged heart. Another name for an enlarged heart is cardiomegaly.

The range of underlying problems that can lead to an enlarged heart may be:

pathological – linked to actual disease of the heart muscle
physiological – linked to other causes that are overworking the heart muscle, such as high blood pressure or thyroid diseases.

Symptoms of an enlarged heart

In some cases, an enlarged heart is asymptomatic (has no symptoms).

When symptoms do occur, it may be because the heart fails to pump blood effectively and this leads to a syndrome known as congestive heart failure. Symptoms may include:

breathing problems
shortness of breath
dizziness
irregular heartbeat (arrhythmia)
heart palpitations
fluid retention.

Causes of enlarged heart

Some of the many causes of enlarged heart include:

coronary artery disease – fatty deposits or plaques build up inside one or more of the coronary (heart) arteries. This constant ‘silting’ is called atherosclerosis and it results in narrowing of the artery. This reduces the oxygen supply, which is the fuel for the pump
high blood pressure (hypertension) – blood pumps with more force than usual through the arteries, which puts strain on the heart. Causes of high blood pressure include obesity and a sedentary lifestyle
idiopathic dilated cardiomyopathy – disease of the heart muscle, the cause of which is unknown. Enlarged or ‘dilated’ heart is one of the most common types of cardiomyopathy. The most common symptom patients get with cardiomyopathy is shortness of breath and swelling of the ankles. Rarer symptoms include dizziness and chest pain
myocarditis – an infection of the heart that is generally caused by a virus. A person may have a viral illness first and later have symptoms of congestive heart failure
heart valve disease – for example, a faulty mitral valve allows blood to flow backwards, which means the affected heart chamber has to contract with more force than usual
cardiac ischaemia – reduced blood flow to the heart. This condition can cause heart pain (angina)
previous heart attack – a weakened heart muscle may enlarge in order to keep up with the demands of pumping blood around the body
thyroid disease – the thyroid gland regulates many metabolic functions. Untreated, a thyroid condition can lead to high blood pressure, high blood cholesterol levels, irregular heartbeat and enlargement of the heart
obesity – carrying too much body fat is a risk factor for high blood pressure, which in turn can cause the heart to enlarge
lack of exercise – leading a sedentary lifestyle is a known risk factor for a range of conditions, including coronary heart disease and high blood pressure
old age – as we get older, our arteries lose some of their elasticity. This ‘stiffening’ of the blood vessels causes high blood pressure, which is a risk factor for enlarged heart.

Diagnosis of enlarged heart

An enlarged heart is diagnosed using a number of tests including:

medical history – including a physical examination
chest x-ray – this allows the doctor to see the overall shape and size of the heart and lungs
echocardiogram – sound waves sent to a special machine present a picture of the beating heart, so the doctor can see the heart as its chambers contract and relax
Doppler study – shows blood flow through the heart valves and evaluates whether the valves are functioning normally
electrocardiogram – measures electrical activity in the heart and can assess heart rhythm and evidence of old infarction or ischaemia.

Treatment of enlarged heart

Treatment depends on the underlying cause but options can include:

medications to stop the heart from enlarging any further
addressing the underlying problem (for example through diet, stopping smoking, exercise and medication to help control high blood pressure, or surgery to replace a faulty heart valve)
regular cardiovascular exercise
adopting a low-fat diet
dietary adjustments to reduce blood cholesterol levels
frequent medical check-ups to make sure the treatments are working.

Global disease estimates in 2005 reported 471,000 ARF cases annually, which largely occurred in children and teens aged 5–15 years, with the prevalence of RHD cases ranging between 15.6–19.6 million (Carapetis, Steer, Mulholland, & Weber, 2005). Approximately 350,000 deaths occurred each year due to either ARF or RHD (Carapetis, Steer, Mulholland, & Weber, 2005). The number of new cases of RHD diagnosed was estimated at 282,000 per year, with approximately 233,000 deaths annually (Carapetis, Steer, Mulholland, & Weber, 2005). Global burden of disease estimates performed in 2010 calculated the number of individuals living with RHD was at least 34.2 million, with 10.1 million disability-adjusted life years lost (de Dassel, Ralph, & Carapetis, 2015). There are challenges in obtaining precise global figures concerning ARF and RHD, with one example being that the diagnosis of ARF remains difficult and problematic across many settings. Improved diagnostic tools and measures are vital, and such efforts would support enhanced global disease estimation efforts (Sheel, Moreland, Fraser, & Carapetis, 2016). It should also be noted that these figures concerning incidence and prevalence are likely to be underestimated, due to variable and insufficient data collection in resource-poor settings, where the rates of ARF and RHD are often highest (Zühlke, et al., 2014). More accurate estimates of RHD prevalence may result from the increased availability of echocardiography, which can detect cardiac damage that is due to RHD more accurately than auscultation (Roberts, et al., 2015).

Risk factors for ARF and RHD include age, gender, and various environmental factors (Carapetis, et al., 2016). In terms of age, ARF largely affects children between the ages of 5 and 14 years, and initial cases of ARF can affect children even younger than this (Lawrence, Carapetis, Griffiths, Edwards, & Condon, 2013; Parnaby & Carapetis, 2010).^. Recurrent episodes generally affect older children and can occur into young adulthood. Because RHD often results from cumulative damage, the peak prevalence of RHD occurs in an individual’s twenties and thirties, though the burden of RHD in children and adolescents remains substantial (Lawrence, Carapetis, Griffiths, Edwards, & Condon, 2013).

While ARF is equally common in both males and females, RHD tends to be more common in females (Lawrence, Carapetis, Griffiths, Edwards, & Condon, 2013; Parnaby & Carapetis, 2010). It is unclear whether this difference in RHD prevalence is due to greater susceptibility to developing autoimmune responses following S. pyogenes infection, or whether social factors such as involvement in child-raising, which may cause increased susceptibility and likelihood of S. pyogenes infections, may combine with reduced access to primary and secondary prevention regimens (Carapetis, et al., 2016). Furthermore, RHD often becomes apparent during pregnancy, because of its associated higher cardiac burden (Carapetis, et al., 2016).

Environmental factors affect the prevalence of ARF by increasing exposure to S. pyogenesinfections. A major environmental factor that increases the likelihood of ARF is household crowding, which facilitates the spread of S. pyogenes infections (Quinn, 1982). In addition, it has been shown that ARF and RHD are more prevalent in rural and remote areas as well as in urban slums, but this likely reflects other risk factors, such as greater household crowding due to low socioeconomic status or limited access to medical resources (Carapetis, et al., 2016). There is also a potential link between insufficient nutrition in childhood and susceptibility to ARF, but it is unclear whether this occurs because insufficient nutrition can increase susceptibility to developing aggressive autoimmune responses to S. pyogenes infection, or whether poor nutrition is connected to household overcrowding and other factors associated with poverty that increase susceptibility to S. pyogenes infection (Steer, Carapetis, Nolan, & Shann, 2002).Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Pathophysiology

The pathogenic mechanisms of ARF are not completely understood. Studies of the pathogenesis of ARF have been constrained by the lack of a highly suitable animal model, although a Lewis rat model of valvulitis and chorea has been used for some time (Quinn, Kosanke, Fischetti, Factor, & Cunningham, 2001; Brimberg, et al., 2012). In order for ARF to occur, it appears that a pharyngeal infection caused by S. pyogenes must occur in a host with a genetic susceptibility to the disease (Denny, Wannamaker, Brink, Rammelkamp, & Custer, 1950; Bryant, Robins-Browne, Carapetis, & Curtis, 2009).

Activation of the innate immune system begins with a pharyngeal infection that leads to the presentation of S. pyogenes antigens to T and B cells. CD4⁺ T cells are activated and production of specific IgG and IgM antibody by B cells ensues (Cunningham, Pathogenesis of group A streptococcal infections, 2000). Tissue injury is mediated through an immune-mediated mechanism that is initiated via molecular mimicry (Guilherme, Kalil, & Cunningham, 2006). Structural similarity between the infectious agent and human proteins leads to the cross-activation of antibodies and/or T cells directed against human proteins (Cunningham, 2000).^.In ARF, this cross-reactive immune response results in the clinical features of rheumatic fever, including carditis, due to antibody binding and infiltration of T cells; transient arthritis, due to the formation of immune complexes; chorea, due to the binding of antibodies to basal ganglia; and skin manifestations, due to a delayed hypersensitivity reaction (Figure 1; Carapetis, et al., 2016).

Congenital heart disease (CHD) is present in about 9 of every 1,000 live-born children. (1)(2)(3)(4)(5) Children with CHD are surviving longer, and better understanding of the long-term complications of CHD is continuously emerging. Hence, it is important to be comfortable with the primary care requirements for these children, including physical manifestations prior to surgery and interventional cardiac catheterizations, as well as those concerning for potential need for reintervention, the latest recommendations for endocarditis prophylaxis, respiratory precautions and immunization considerations, and close monitoring of development and behavior. In this article, we will discuss the common types of cyanotic (“blue”) and acyanotic (“pink”) CHD and the role of the primary care physician in the health care of these children before and after surgery and interventional cardiac catheterizations.

Pediatric and young adult patients with congenital heart disease (CHD) represent a population with unique medical issues, who pose a challenge to the primary care physician (PCP). Advances in pediatric cardiology and cardiac surgery have resulted in an increasing number of survivors with CHD.1 As a result, PCPs are now more likely to encounter patients with varying severity of CHD in their daily practice. In this chapter, we aim to discuss some common pediatric issues that may be encountered while taking care of this subset of patients.

Oral health is an integral part of general health, and according to the Centers for Disease Control and Prevention, dental caries are the most prevalent infectious disease among U.S. children.2 More than 40% of children have tooth decay by the time they reach kindergarten, and more than 52 million hours of school are lost each year because of dental problems.2,3 Oral disease poses a significant burden in patients with CHD, not only by increasing the risk of acute and subacute endocarditis, but also by exposing these children to the risk associated with oral procedures. The morbidity and mortality of endocarditis is significant.4,5 One important objective of the PCP caring for patients with CHD is to guide families to prevent oral pathology.4,5 Oral disease increases morbidity and mortality in children with CHD by different mechanisms. The mouth is a portal of entry for microbial infection. Bacteremia may cause injury by directly damaging the epithelium and indirectly by generating an inflammatory response. Through these mechanisms and potentially others, periodontitis may further increase the known risk of endocarditis. More recently, an association between periodontitis and increasing atherogenesis and thromboembolic phenomena has been described in adults.4 In more severe cases, oral disease may also compromise nutrition, which in turn may have a deleterious effect on the course of the CHD.Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

Children with CHD have variable cardiovascular reserve; those with more tenuous hemodynamics are at higher risk of complications during dental intervention. For example, some local anesthetics with vasoconstrictors like epinephrine may be contraindicated in patients with refractory dysrhythmias. Some patients with CHD, such as patients with prosthetic valves or implanted devices, may require anticoagulation with agents like warfarin or aspirin. Stopping these agents to prevent severe bleeding may be appropriate during the much needed dental procedure but may increase the risk of valve or intracardiac thrombosis. Alternative strategies such as transition to heparin or enoxaparin and careful control of the coagulation profile before surgical intervention may be indicated. Consultation with hematology and coagulation experts may be essential in some of the complex cases. Stress and pain secondary to the dental procedure may generate catecholamine release enough to compromise hemodynamic stability. In the attempt to limit stress and pain in this vulnerable population,

The heart murmur is a subject that has had countless volumes of literature written about it and a brief appraisal of this evidence base suggests that the single most important consideration is to sort those that are clinically significant from those that are of no clinical significance at all. (Farrer, K F M et al 2003)

In broad terms, a heart murmur is an audible indication that there is turbulence in the normally laminar blood flow through the heart. It is often discovered during a routine examination and is more often than not completely asymptomatic when found. (Biancaniello, T. 2005).

In any discussion relating to the actual physical needs of the disorder one must first take account of the likelihood of any given heart murmur being associated with a degree of pathology. The evidence base (Green & Britten 1998) suggests that even in the first group ( those admitted for investigation) over half will be found to have no disease present (Newburger JW et al 1993).

It is therefore clear that in this group (from a medical point of view) nothing more than simple reassurance will be required. This comment should not be taken lightly nor undervalued as the majority of adolescents (and presumably their parents) will probably have been mortified by the discovery of a heart murmur. The majority of lay people will immediately associate the diagnosis with serious pathology, particularly if this is also associated with the necessity to be admitted into hospital for further investigation. (Gaskin, P. R. A et al 2000).

A simple comment that “nothing has been found” is entirely inappropriate in this group. The professional nurse will consider it is part of their remit to spend some time with both the patient and their family and explain the true significance of an asymptomatic heart murmur and the fact that there need be no restrictions on activity, as it probably will not be otherwise understood. (Hogston &. Simpson, 2002)

With regard to the second group of patients who are in hospital for assessment and possible treatment, they will almost universally be concerned and scared of the high technology environment into which they suddenly find themselves propelled. (Birkebæk, N. H et al 1999).

The empathetic nurse will clearly recognise this in both the patient and their parents and will find the time to discuss the particular case with them, to sensitively explore their particular worries and to adopt an approach of empowerment and education by means of thorough explanation of the areas that they raise (Howe & Anderson 2003)

Clearly the management of the particular patient with a heart murmur will depend primarily on the actual pathology found. In an essay such as this it is neither practical nor appropriate to cover every eventuality, but in broad terms, the majority of heart murmurs that are found to be associated with pathology will have a diagnosis of either a valvular disorder or some form of septal defect. In modern practice, both eventualities are considered almost universally treatable and almost routine. (McDonald, I G et al 1999),

The nursing input in these cases must be not only to educate the patient and their parents but possibly also act as their advocate with the medical or surgical teams. (Marks-Moran & Rose 1996)

It follows form the discussions presented here that perhaps the most important general skill that is applicable to virtually all cases of the hospitalised adolescent with a heart murmur is the skill of communication. It follows that this requires the nurse to have a firm grasp of the pathophysiology of the situation in order to be able to communicate clearly and authoritatively with the patient. It also requires a positive realisation that the patient, and their parents, will have a large number of (probably) unspoken concerns which they would ideally like a healthcare professional to address. It therefore requires some sympathetic and empathetic enquiries to address this need in the patient in order to achieve the best outcome for them. (Newell and Simon. 1992) Caring for Hospitalized Adolescents with Heart Murmur Essay Paper

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