Rheumatic Heart Disease in Children and Adolescents

Introduction to Rheumatic Heart Disease in Children

Rheumatic Heart Disease (RHD) is a serious cardiovascular condition resulting from acute rheumatic fever (ARF), primarily affecting children and young adults in developing countries. It remains a significant global health concern, particularly in low- and middle-income regions.

  • RHD is the most common acquired heart disease in children worldwide.
  • It affects approximately 33 million people globally.
  • The disease is most prevalent in Sub-Saharan Africa, South Asia, and among indigenous populations in developed countries.
  • RHD accounts for about 300,000 deaths annually, with many of these occurring in children and young adults.

Understanding RHD is crucial for pediatricians, cardiologists, and public health professionals to implement effective prevention strategies and manage affected children appropriately.

Etiology of Rheumatic Heart Disease

RHD is a sequela of acute rheumatic fever (ARF), which is triggered by an autoimmune response to group A β-hemolytic streptococcal (GAS) infection, typically of the throat.

Key Points:

  • Causative Agent: Group A Streptococcus (Streptococcus pyogenes)
  • Primary Infection Site: Pharynx (strep throat)
  • Risk Factors:
    • Poverty and overcrowding
    • Limited access to healthcare
    • Genetic susceptibility
    • Repeated GAS infections

The progression from GAS infection to ARF and subsequently to RHD involves a complex interplay of bacterial virulence factors, host immune response, and genetic predisposition.

Pathophysiology of Rheumatic Heart Disease

The pathophysiology of RHD is characterized by an abnormal immune response to GAS antigens, leading to inflammation and damage to various tissues, particularly the heart valves.

Key Mechanisms:

  1. Molecular Mimicry:
    • GAS antigens share structural similarities with human proteins (e.g., cardiac myosin)
    • Cross-reactive antibodies attack host tissues
  2. Inflammatory Response:
    • T-cell mediated inflammation
    • Cytokine release (TNF-α, IL-1, IL-6)
  3. Valve Damage:
    • Initial inflammation leads to valve leaflet edema and cellular infiltration
    • Repeated episodes cause progressive scarring and deformity
    • Most commonly affects mitral valve, followed by aortic valve

The chronic inflammatory process results in fibrosis, calcification, and distortion of valve leaflets, leading to stenosis and/or regurgitation.

Clinical Manifestations of Rheumatic Heart Disease in Children

The clinical presentation of RHD in children can vary from asymptomatic valve lesions to severe heart failure. Symptoms typically develop years after the initial ARF episode.

Common Presentations:

  1. Asymptomatic Valve Lesions:
    • Detected on routine examination or screening echocardiography
    • May have a heart murmur without symptoms
  2. Mitral Valve Disease:
    • Mitral regurgitation: Dyspnea, fatigue, palpitations
    • Mitral stenosis: Exertional dyspnea, orthopnea, hemoptysis
  3. Aortic Valve Disease:
    • Aortic regurgitation: Exertional dyspnea, palpitations, chest pain
    • Aortic stenosis: Syncope, angina, exertional dyspnea
  4. Heart Failure:
    • Dyspnea, orthopnea, peripheral edema
    • Hepatomegaly, ascites in advanced cases
  5. Atrial Fibrillation:
    • Palpitations, irregular pulse
    • Increased risk of thromboembolism

It's important to note that children may not always present with typical symptoms, and a high index of suspicion is necessary in endemic areas.

Diagnosis of Rheumatic Heart Disease in Children

Accurate and early diagnosis of RHD is crucial for effective management and prevention of complications. The diagnosis involves a combination of clinical assessment, laboratory tests, and imaging studies.

Diagnostic Approach:

  1. Clinical Evaluation:
    • Detailed history (including history of ARF)
    • Physical examination (heart murmurs, signs of heart failure)
  2. Laboratory Tests:
    • Acute phase reactants (ESR, CRP) - elevated in active carditis
    • Anti-streptolysin O (ASO) titers - evidence of recent streptococcal infection
    • Throat culture - to identify GAS
  3. Electrocardiogram (ECG):
    • May show prolonged PR interval, arrhythmias
  4. Chest X-ray:
    • Cardiomegaly, pulmonary congestion in advanced cases
  5. Echocardiography:
    • Gold standard for diagnosis and assessment of valve lesions
    • Evaluates valve morphology, function, and hemodynamics
    • Essential for screening and follow-up

Diagnostic Criteria:

The World Heart Federation (WHF) criteria for echocardiographic diagnosis of RHD are widely used:

  • Definite RHD:
    • Pathological mitral regurgitation and at least two morphological features of RHD of the mitral valve
    • Mitral stenosis mean gradient ≥ 4 mmHg
    • Pathological aortic regurgitation and at least two morphological features of RHD of the aortic valve
    • Borderline disease of both the aortic and mitral valve
  • Borderline RHD:
    • At least two morphological features of RHD of the mitral valve without pathological mitral regurgitation or mitral stenosis
    • Pathological mitral regurgitation
    • Pathological aortic regurgitation

Early diagnosis through echocardiographic screening programs has shown promise in identifying subclinical RHD, allowing for early intervention and prevention of progression.

Treatment of Rheumatic Heart Disease in Children

The management of RHD in children involves a multidisciplinary approach aimed at preventing disease progression, managing symptoms, and addressing complications. Treatment strategies depend on the severity of valve lesions and the presence of heart failure.

Key Components of Treatment:

  1. Secondary Prophylaxis:
    • Long-term penicillin prophylaxis (typically intramuscular benzathine penicillin G every 3-4 weeks)
    • Crucial for preventing recurrent ARF and further valve damage
    • Duration depends on severity of RHD and risk factors
  2. Medical Management:
    • Heart Failure Treatment:
      • Diuretics (e.g., furosemide)
      • ACE inhibitors or ARBs
      • Beta-blockers in selected cases
      • Digoxin for rate control in atrial fibrillation
    • Anticoagulation:
      • Warfarin for atrial fibrillation or mechanical valve prostheses
  3. Surgical Interventions:
    • Valve Repair:
      • Preferred option, especially in children
      • Mitral valve repair techniques (e.g., commissurotomy, chordal shortening)
    • Valve Replacement:
      • Mechanical or bioprosthetic valves
      • Considered when repair is not feasible
    • Timing of surgery is crucial - balancing the risks of early intervention against the benefits of preserving ventricular function
  4. Percutaneous Interventions:
    • Balloon Mitral Valvuloplasty:
      • For suitable cases of mitral stenosis
      • Can delay the need for surgical intervention

Follow-up and Monitoring:

  • Regular echocardiographic assessment (frequency based on severity)
  • Monitoring of anticoagulation therapy
  • Assessment of growth and development
  • Psychosocial support and education for patients and families

Treatment decisions should be individualized based on the patient's age, valve lesion severity, ventricular function, and available resources. A collaborative approach involving pediatric cardiologists, cardiac surgeons, and rheumatologists is essential for optimal outcomes.

Prevention of Rheumatic Heart Disease in Children

Prevention is a cornerstone in the management of RHD, encompassing both primary and secondary prevention strategies. Effective prevention can significantly reduce the burden of RHD in endemic areas.

Primary Prevention:

  • Aims to prevent the initial attack of ARF
  • Key strategies:
    • Prompt diagnosis and treatment of GAS pharyngitis
      • Penicillin (oral or intramuscular) is the first-line treatment
      • Amoxicillin or macrolides for penicillin-allergic patients
    • Improving access to healthcare in underserved areas
    • Public health measures to reduce overcrowding and improve sanitation
    • Education about the importance of treating sore throats

Secondary Prevention:

  • Aims to prevent recurrence of ARF in those with a history of ARF or established RHD
  • Key components:
    • Long-term antibiotic prophylaxis
      • Intramuscular benzathine penicillin G every 3-4 weeks (preferred method)
      • Oral penicillin V or sulfadiazine as alternatives
    • Duration of prophylaxis:
      • For patients without carditis: at least 5 years or until 21 years of age, whichever is longer
      • For patients with carditis: at least 10 years or until 21 years of age, whichever is longer
      • Lifelong prophylaxis may be considered in high-risk cases

Tertiary Prevention:

  • Focuses on preventing complications in those with established RHD
  • Strategies include:
    • Regular medical follow-up and echocardiography
    • Timely interventions (medical or surgical) to prevent heart failure
    • Anticoagulation for atrial fibrillation
    • Endocarditis prophylaxis in high-risk patients

Population-based Strategies:

  • Echocardiographic screening programs in endemic areas
  • Integration of RHD prevention into primary health care systems
  • Development of RHD registries for better surveillance and management
  • Community education and awareness programs

Effective prevention of RHD requires a comprehensive approach involving healthcare providers, public health officials, and policymakers. Implementing these strategies can significantly reduce the incidence and progression of RHD in children.

Prognosis of Rheumatic Heart Disease in Children

The prognosis of Rheumatic Heart Disease (RHD) in children varies widely depending on several factors, including the severity of valve lesions, adherence to secondary prophylaxis, access to healthcare, and timely interventions. Understanding the prognosis is crucial for clinicians to guide management decisions and counsel patients and their families.

Factors Influencing Prognosis:

  1. Severity of Valve Lesions:
    • Mild cases with good adherence to prophylaxis may have an excellent prognosis
    • Severe valve lesions, especially those involving multiple valves, carry a poorer prognosis
  2. Age at Diagnosis:
    • Earlier onset generally indicates more aggressive disease and potentially worse outcomes
    • Children diagnosed before age 10 may have more rapid progression of valve disease
  3. Adherence to Secondary Prophylaxis:
    • Consistent adherence significantly reduces the risk of ARF recurrence and disease progression
    • Poor adherence is associated with worse outcomes and increased risk of complications
  4. Access to Healthcare:
    • Regular follow-up and access to advanced cardiac care improve long-term outcomes
    • Limited access to healthcare in resource-poor settings can lead to delayed interventions and poorer prognosis
  5. Presence of Complications:
    • Development of heart failure, pulmonary hypertension, or atrial fibrillation worsens prognosis
    • Infective endocarditis can lead to rapid deterioration and poor outcomes

Long-term Outcomes:

  • Mortality:
    • 5-year mortality rates vary widely, from less than 5% in mild cases to over 30% in severe cases with complications
    • Mortality is highest in the first year after diagnosis of severe RHD
  • Morbidity:
    • Chronic heart failure affects 30-40% of patients with severe RHD
    • Atrial fibrillation develops in 15-30% of patients over long-term follow-up
    • Stroke risk is increased, especially in those with atrial fibrillation
  • Quality of Life:
    • Many children with well-managed RHD can lead normal or near-normal lives
    • Severe cases may face limitations in physical activities and require frequent hospitalizations

Prognostic Indicators:

  • Echocardiographic Findings:
    • Left ventricular ejection fraction < 50% is associated with poorer outcomes
    • Severe pulmonary hypertension (systolic pulmonary artery pressure > 50 mmHg) indicates advanced disease
  • Biomarkers:
    • Elevated BNP or NT-proBNP levels correlate with worse prognosis
    • Persistently elevated inflammatory markers may indicate ongoing subclinical carditis

Special Considerations:

  • Pregnancy:
    • RHD complicates 0.3-3.5% of pregnancies in endemic areas
    • Increased risk of cardiac decompensation, especially in those with severe mitral stenosis
    • Careful preconception counseling and management is crucial
  • Transition to Adult Care:
    • Successful transition from pediatric to adult cardiac care is essential for long-term outcomes
    • Requires coordinated care and patient education to ensure continued adherence to management plans

Future Perspectives:

  • Improved Screening and Early Detection:
    • Echocardiographic screening programs may lead to earlier diagnosis and intervention
    • Potential for improved outcomes with early initiation of secondary prophylaxis
  • Novel Therapies:
    • Research into immunomodulatory therapies may alter the natural history of RHD
    • Advances in valve repair techniques and transcatheter interventions may improve surgical outcomes
  • Global RHD Control:
    • Implementation of comprehensive RHD control programs in endemic areas may significantly improve overall prognosis
    • Potential for RHD elimination in some regions with sustained public health efforts

In conclusion, while RHD remains a significant cause of morbidity and mortality in children worldwide, early diagnosis, consistent secondary prophylaxis, and timely interventions can substantially improve prognosis. Ongoing research and global health initiatives offer hope for better outcomes and potential disease control in the future.

Mitral Regurgitation in Rheumatic Heart Disease

Mitral regurgitation (MR) is the most common valvular lesion in children with RHD, often occurring early in the disease process.

Pathophysiology:

  • Acute phase: Valve leaflet edema and chordal inflammation
  • Chronic phase: Leaflet thickening, commissural fusion, chordal shortening and fusion
  • Results in inadequate leaflet coaptation during systole, causing backward flow into the left atrium

Clinical Presentation:

  • Asymptomatic in mild cases
  • Exertional dyspnea and fatigue in moderate to severe cases
  • Palpitations due to atrial enlargement and potential atrial fibrillation
  • Signs of heart failure in advanced cases

Diagnosis:

  • Auscultation: Holosystolic murmur at the apex, radiating to the axilla
  • Echocardiography:
    • Gold standard for diagnosis and severity assessment
    • Assesses leaflet morphology, regurgitant jet, and left atrial/ventricular size
  • Chest X-ray: Enlarged left atrium and left ventricle in chronic cases
  • ECG: May show left atrial enlargement and left ventricular hypertrophy

Management:

  • Medical management:
    • Secondary prophylaxis with penicillin
    • Diuretics and afterload reduction in symptomatic cases
  • Surgical intervention:
    • Mitral valve repair preferred over replacement, especially in children
    • Timing based on symptoms, left ventricular function, and chamber dimensions

Prognosis:

  • Generally good with early detection and appropriate management
  • Risk of progression to severe MR and heart failure if left untreated
  • Outcomes after valve repair generally better than replacement in children

Mitral Stenosis in Rheumatic Heart Disease

Mitral stenosis (MS) is a common manifestation of chronic RHD, typically developing years after the initial rheumatic fever episode.

Pathophysiology:

  • Progressive leaflet thickening and calcification
  • Commissural fusion and chordal shortening
  • Results in obstruction to left atrial emptying, leading to increased left atrial pressure

Clinical Presentation:

  • Often asymptomatic in early stages
  • Exertional dyspnea and fatigue as stenosis progresses
  • Hemoptysis due to rupture of bronchial veins
  • Atrial fibrillation in advanced cases

Diagnosis:

  • Auscultation: Low-pitched diastolic rumble at the apex, accentuated first heart sound
  • Echocardiography:
    • Key diagnostic tool
    • Assesses valve area, gradient across the valve, and pulmonary artery pressure
  • Chest X-ray: May show left atrial enlargement and pulmonary congestion
  • ECG: Left atrial enlargement, possible right ventricular hypertrophy in advanced cases

Management:

  • Medical management:
    • Secondary prophylaxis
    • Diuretics for symptomatic relief
    • Rate control and anticoagulation for atrial fibrillation
  • Interventional procedures:
    • Percutaneous balloon mitral valvuloplasty: Preferred initial intervention in suitable cases
    • Surgical commissurotomy or valve replacement if balloon valvuloplasty is not feasible

Prognosis:

  • Variable, depending on severity and timing of intervention
  • Good outcomes with early intervention, especially with balloon valvuloplasty
  • Risk of complications including pulmonary hypertension and right heart failure if left untreated

Aortic Regurgitation in Rheumatic Heart Disease

Aortic regurgitation (AR) is less common than mitral valve disease in children with RHD but can occur either in isolation or in combination with mitral valve involvement.

Pathophysiology:

  • Leaflet thickening and retraction
  • Commissural fusion leading to incomplete closure of the valve
  • Results in diastolic backflow from the aorta into the left ventricle

Clinical Presentation:

  • Often asymptomatic in early stages
  • Exertional dyspnea and fatigue in more advanced cases
  • Palpitations and angina in severe cases
  • Signs of left ventricular volume overload

Diagnosis:

  • Auscultation: Early diastolic decrescendo murmur best heard at the left sternal border
  • Echocardiography:
    • Assesses severity of regurgitation, aortic valve morphology, and left ventricular size and function
    • Color Doppler demonstrates the regurgitant jet
  • Chest X-ray: May show left ventricular enlargement and ascending aorta dilatation
  • ECG: Left ventricular hypertrophy in chronic cases

Management:

  • Medical management:
    • Secondary prophylaxis
    • Afterload reduction with ACE inhibitors or ARBs in significant AR
  • Surgical intervention:
    • Aortic valve repair when feasible, especially in children
    • Aortic valve replacement if repair is not possible
    • Timing based on symptoms, left ventricular size, and function

Prognosis:

  • Generally good with appropriate management and timely intervention
  • Risk of progressive left ventricular dilatation and dysfunction if left untreated
  • Outcomes after valve repair generally better than replacement in children, but long-term durability can be a concern

Aortic Stenosis in Rheumatic Heart Disease

Aortic stenosis (AS) is less common than aortic regurgitation in RHD but can occur, especially in long-standing disease.

Pathophysiology:

  • Progressive leaflet thickening and calcification
  • Commissural fusion leading to narrowing of the valve orifice
  • Results in obstruction to left ventricular outflow

Clinical Presentation:

  • Often asymptomatic until stenosis becomes severe
  • Classic triad of symptoms in advanced cases:
    • Exertional dyspnea
    • Angina
    • Syncope or presyncope
  • Reduced exercise tolerance

Diagnosis:

  • Auscultation: Harsh systolic ejection murmur, best heard at the right upper sternal border
  • Echocardiography:
    • Key diagnostic tool
    • Assesses valve morphology, aortic valve area, and gradient across the valve
    • Evaluates left ventricular hypertrophy and function
  • Chest X-ray: May show left ventricular hypertrophy and post-stenotic dilatation of the ascending aorta
  • ECG: Left ventricular hypertrophy, possible ST-segment depression and T-wave inversion

Management:

  • Medical management:
    • Secondary prophylaxis
    • Management of symptoms (e.g., diuretics for congestion)
    • Avoidance of strenuous physical activity in severe AS
  • Interventional procedures:
    • Balloon aortic valvuloplasty: May be considered as a bridge to surgery in children
    • Surgical aortic valve replacement: Definitive treatment for severe AS
    • Ross procedure (pulmonary autograft) may be preferred in children and young adults

Prognosis:

  • Variable, depending on severity and timing of intervention
  • Risk of sudden cardiac death in severe, symptomatic AS
  • Generally good outcomes with timely surgical intervention
  • Need for long-term follow-up and possible reintervention, especially with bioprosthetic valves or Ross procedure

Tricuspid Valve Disease in Rheumatic Heart Disease

Tricuspid valve involvement in RHD is less common than left-sided valve disease and usually occurs in conjunction with mitral and/or aortic valve disease.

Pathophysiology:

  • Direct rheumatic involvement of the tricuspid valve
  • Secondary tricuspid regurgitation due to right ventricular dilatation from left-sided valve disease
  • Leaflet thickening, commissural fusion, and chordal shortening can occur

Clinical Presentation:

  • Often overshadowed by symptoms of left-sided valve disease
  • Signs of right heart failure in advanced cases:
    • Peripheral edema
    • Hepatomegaly
    • Ascites
  • Fatigue and reduced exercise tolerance

Diagnosis:

  • Auscultation:
    • Tricuspid regurgitation: Holosystolic murmur at the left lower sternal border, increasing with inspiration
    • Tricuspid stenosis: Diastolic rumble at the left lower sternal border (rare)
  • Echocardiography:
    • Primary diagnostic tool
    • Assesses tricuspid valve morphology, severity of regurgitation or stenosis
    • Evaluates right ventricular size and function
  • Chest X-ray: May show right atrial enlargement and prominent central pulmonary arteries
  • ECG: Right atrial enlargement, possible right ventricular hypertrophy

Management:

  • Medical management:
    • Secondary prophylaxis
    • Diuretics for symptomatic relief of right heart failure
  • Surgical intervention:
    • Often addressed during surgery for left-sided valve disease
    • Tricuspid valve repair preferred over replacement when possible
    • Timing based on severity of symptoms and right ventricular function

Prognosis:

  • Generally determined by the severity of associated left-sided valve disease
  • Isolated tricuspid valve disease carries a better prognosis than combined valve lesions
  • Outcomes after tricuspid valve repair are generally good, with low reoperation rates

Multiple Valve Disease in Rheumatic Heart Disease

Multiple valve involvement is common in RHD, particularly in advanced or long-standing disease. This complex presentation poses unique challenges in management and prognosis.

Pathophysiology:

  • Simultaneous or sequential involvement of multiple valves due to recurrent rheumatic inflammation
  • Most common combination: Mitral and aortic valve disease
  • Tricuspid valve involvement often secondary to left-sided heart disease
  • Complex hemodynamic interactions between affected valves

Clinical Presentation:

  • Symptoms may be dominated by the most severely affected valve
  • Common symptoms include:
    • Dyspnea on exertion progressing to orthopnea and paroxysmal nocturnal dyspnea
    • Fatigue and exercise intolerance
    • Palpitations due to atrial arrhythmias
    • Signs of both left and right heart failure in advanced cases
  • Physical examination may reveal multiple murmurs, which can be challenging to differentiate

Diagnosis:

  • Echocardiography:
    • Cornerstone of diagnosis
    • Assesses morphology and function of all valves
    • Evaluates severity of each lesion and their combined hemodynamic effects
    • Measures chamber sizes and ventricular function
  • Transesophageal echocardiography (TEE) may be necessary for more detailed assessment
  • Cardiac catheterization:
    • May be required in complex cases to assess hemodynamics
    • Helps in surgical planning by evaluating coronary arteries in older patients
  • Cardiac MRI:
    • Provides detailed assessment of ventricular volumes and function
    • Useful for quantifying regurgitant volumes in multiple valve disease

Management:

  • Medical management:
    • Secondary prophylaxis is crucial to prevent disease progression
    • Heart failure management with diuretics, ACE inhibitors, and beta-blockers as appropriate
    • Rhythm control and anticoagulation for atrial fibrillation
  • Surgical intervention:
    • Timing is critical and often complex, balancing risks and benefits
    • Options include:
      • Multi-valve repair
      • Combination of repair and replacement
      • Multi-valve replacement
    • Valve repair is preferred when feasible, especially in children
    • Decision-making should involve a multidisciplinary heart team
  • Percutaneous interventions:
    • Balloon valvuloplasty may be considered for isolated mitral or aortic stenosis in suitable cases
    • Limited role in multiple valve disease, mainly used as a bridge to surgery

Prognosis:

  • Generally poorer than single valve disease due to complex hemodynamics
  • Factors affecting prognosis include:
    • Severity and combination of valve lesions
    • Ventricular function at the time of intervention
    • Presence of pulmonary hypertension
    • Timing and success of surgical intervention
  • Operative risk is higher in multiple valve surgery compared to single valve procedures
  • Long-term outcomes are variable, with many patients requiring reintervention over time

Special Considerations in Children:

  • Growth and development:
    • Multiple valve disease can significantly impact a child's growth and development
    • Timing of intervention must balance the need for definitive treatment with allowing for somatic growth
  • Valve choices:
    • Preference for valve repair over replacement whenever possible
    • If replacement is necessary, options include:
      • Mechanical valves: Durable but require lifelong anticoagulation
      • Bioprosthetic valves: Avoid anticoagulation but have limited durability in children
      • Ross procedure for aortic valve disease: Allows for growth but adds complexity in multiple valve disease
  • Follow-up:
    • Lifelong, specialized follow-up is essential
    • Regular echocardiographic assessment to monitor disease progression and intervention outcomes
    • Careful monitoring of anticoagulation if mechanical valves are used

Future Perspectives:

  • Advances in surgical techniques:
    • Minimally invasive and robotic approaches may reduce surgical morbidity
    • Tissue engineering and bioengineered valves hold promise for growing valves in children
  • Percutaneous therapies:
    • Development of transcatheter technologies for multiple valve interventions
    • May offer less invasive options for high-risk patients
  • Global RHD control:
    • Improved prevention strategies may reduce the incidence of advanced, multiple valve RHD
    • Enhanced access to early surgical intervention in endemic areas could improve overall outcomes

Management of multiple valve disease in RHD remains one of the most challenging aspects of pediatric cardiology and cardiac surgery. A tailored approach, considering the child's age, growth potential, and the specific combination of valve lesions, is crucial for optimal outcomes. Ongoing research and technological advancements offer hope for improved management strategies in the future.



Objective QnA: Rheumatic Heart Disease in Children and Adolescents
  1. Question: What is the causative organism of rheumatic fever and rheumatic heart disease? Answer: Group A beta-hemolytic Streptococcus
  2. Question: Which age group is most commonly affected by acute rheumatic fever? Answer: 5-15 years old
  3. Question: What is the most common and specific manifestation of rheumatic heart disease? Answer: Mitral valve stenosis
  4. Question: Which diagnostic criteria are used for the diagnosis of acute rheumatic fever? Answer: Jones Criteria (revised)
  5. Question: What is the recommended first-line treatment for acute rheumatic fever? Answer: Penicillin
  6. Question: Which cardiac valve is most commonly affected in rheumatic heart disease? Answer: Mitral valve
  7. Question: What is the role of echocardiography in rheumatic heart disease? Answer: To assess valve morphology and function, and monitor disease progression
  8. Question: What is the recommended duration of secondary prophylaxis in rheumatic heart disease without carditis? Answer: 5 years or until age 21, whichever is longer
  9. Question: Which major manifestation of acute rheumatic fever is considered pathognomonic? Answer: Sydenham's chorea
  10. Question: What is the most common arrhythmia associated with rheumatic heart disease? Answer: Atrial fibrillation
  11. Question: Which blood test is used to support the diagnosis of a recent streptococcal infection? Answer: Anti-streptolysin O (ASO) titer
  12. Question: What is the recommended interval for benzathine penicillin G injections for secondary prophylaxis? Answer: Every 3-4 weeks
  13. Question: Which complication of rheumatic heart disease is associated with left atrial enlargement? Answer: Thromboembolic events
  14. Question: What is the typical heart murmur heard in rheumatic mitral stenosis? Answer: Diastolic rumble at the apex
  15. Question: Which inflammatory marker is elevated in acute rheumatic fever? Answer: Erythrocyte sedimentation rate (ESR)
  16. Question: What is the recommended first-line treatment for Sydenham's chorea? Answer: Valproic acid
  17. Question: Which cardiac complication of rheumatic heart disease may require urgent intervention? Answer: Acute pulmonary edema due to severe mitral stenosis
  18. Question: What is the role of anti-inflammatory treatment in acute rheumatic fever? Answer: To reduce inflammation and prevent valve damage
  19. Question: Which imaging modality is used to assess for subclinical carditis in acute rheumatic fever? Answer: Echocardiography
  20. Question: What is the recommended duration of secondary prophylaxis in rheumatic heart disease with severe valve disease? Answer: Lifelong
  21. Question: Which non-cardiac manifestation of acute rheumatic fever affects the skin? Answer: Erythema marginatum
  22. Question: What is the most common valvular lesion in rheumatic heart disease? Answer: Mitral regurgitation
  23. Question: Which complication of rheumatic heart disease may require anticoagulation? Answer: Atrial fibrillation
  24. Question: What is the role of balloon valvuloplasty in rheumatic heart disease? Answer: To treat severe mitral stenosis in suitable patients
  25. Question: Which joint manifestation is characteristic of acute rheumatic fever? Answer: Migratory polyarthritis
  26. Question: What is the recommended treatment for recurrent acute rheumatic fever in a penicillin-allergic patient? Answer: Erythromycin or azithromycin
  27. Question: Which cardiac finding on auscultation is associated with rheumatic aortic regurgitation? Answer: Early diastolic decrescendo murmur at the left sternal border
  28. Question: What is the role of C-reactive protein (CRP) in diagnosing acute rheumatic fever? Answer: To support the diagnosis and monitor disease activity
  29. Question: Which echocardiographic finding is consistent with rheumatic mitral valve disease? Answer: Fish-mouth appearance of the mitral valve in diastole
  30. Question: What is the most common cause of death in patients with rheumatic heart disease? Answer: Heart failure


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