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Cardiovascular System

Rheumatic Heart Disease (RHD)

Chronic heart valve damage resulting from one or more episodes of Acute Rheumatic Fever (ARF). The inflammation during ARF causes scarring and deformity of heart valves, most commonly the mitral and aortic valves, leading to stenosis (narrowing) and/or regurgitation (leakage).

Also known as: RHD, Rheumatic Valvular Disease

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Referral and warning signs

When to refer

Referral to cardiologist (H/RR/Specialist Centre) for: Initial diagnosis and assessment of severity via echocardiogram. Management of moderate/severe RHD. Development of symptoms (HF, AF, embolism). Consideration for surgical or interventional procedures (valve repair/replacement, PMBC). Management of complications.

Red flags

  • Development or worsening of heart failure symptoms (dyspnoea, oedema)
  • New onset atrial fibrillation
  • Symptoms of stroke or systemic embolism
  • Syncope or pre-syncope (especially with aortic stenosis)
  • Signs of infective endocarditis (fever, new murmur, emboli)

Signs and symptoms

  • Often asymptomatic for years after ARF.
  • Symptoms develop gradually as valve damage progresses:
  • - Dyspnoea on exertion, progressing to orthopnea, PND, and rest dyspnoea (signs of heart failure).
  • - Fatigue, reduced exercise tolerance.
  • - Palpitations (often due to atrial fibrillation).
  • - Chest pain (angina-like, especially with aortic stenosis).
  • - Syncope or pre-syncope (especially with aortic stenosis).
  • - Symptoms of systemic embolism (stroke, limb ischaemia) if atrial fibrillation present.
  • - Haemoptysis (coughing blood - esp. with severe mitral stenosis).
  • Clinical Signs:
  • - Characteristic heart murmurs depending on valve(s) affected (e.g., mid-diastolic murmur of mitral stenosis, pansystolic murmur of mitral regurgitation, ejection systolic/early diastolic murmur of aortic stenosis/regurgitation).
  • - Loud S1 (mitral stenosis), Opening snap (mitral stenosis).
  • - Signs of heart failure (raised JVP, oedema, hepatomegaly, lung crepitations).
  • - Signs of atrial fibrillation (irregularly irregular pulse).
  • - Malar flush (mitral stenosis).

Risk factors

  • History of Acute Rheumatic Fever (ARF) - single or multiple episodes.
  • Inadequate or lack of secondary prophylaxis after ARF.
  • Living in endemic areas with high rates of GAS infection and ARF.
  • Factors predisposing to ARF (poverty, overcrowding).

Diagnosis

Clinical assessment

Suspected based on history of ARF (often remote/unrecognized) and presence of characteristic heart murmurs on examination, often with symptoms/signs of heart failure or atrial fibrillation. Differentiate murmurs from innocent murmurs or other causes of valvular disease.

Investigations

  • Echocardiogram: Definitive diagnostic tool. Confirms valvular lesions (stenosis, regurgitation, mixed), assesses severity, measures chamber sizes, estimates pulmonary pressures, assesses ventricular function. Essential for diagnosis and management decisions.
  • Chest X-ray: Shows specific chamber enlargement (e.g., left atrial enlargement in mitral disease), cardiomegaly, pulmonary vascular congestion.
  • ECG: May show chamber hypertrophy (e.g., LAE, LVH, RVH), P mitrale (mitral stenosis), signs of atrial fibrillation, or axis deviation.
  • Cardiac Catheterization: Less commonly needed for diagnosis now with advanced echo, but may be used to assess haemodynamic severity or coronary arteries pre-operatively.

Diagnostic criteria

Echocardiographic confirmation of characteristic rheumatic valve damage (thickening, calcification, restricted motion, commissural fusion) leading to stenosis and/or regurgitation, often in a patient with a history suggestive of ARF or from an endemic area.

Management

Non-pharmacological management

  • Regular clinical follow-up and echocardiographic surveillance (frequency depends on severity).
  • Lifestyle advice similar to heart failure (salt restriction if HF present).
  • Management of complications (Heart Failure - see CV003; Atrial Fibrillation - see CV005).
  • Surgical/Interventional Management (Referral to Cardiac Centre):
  • - Valve Repair or Replacement: Indicated for severe symptomatic valvular disease or asymptomatic severe disease with specific criteria (e.g., LV dilatation/dysfunction).
  • - Percutaneous Mitral Balloon Commissurotomy (PMBC): For suitable cases of pure mitral stenosis.

Pharmacological treatment

  • Secondary Prophylaxis against ARF Recurrence: Essential lifelong management. Benzathine Penicillin G 1.2 MU IM every 3-4 weeks (or oral Pen V/Erythromycin alternatives). See ARF section (CV009) for duration details (often lifelong if significant RHD).
  • Management of Heart Failure: Diuretics, ACE inhibitors/ARBs, Beta-blockers (use cautiously in severe stenosis), MRAs as indicated (see CV003).
  • Management of Atrial Fibrillation: Rate control (Beta-blockers, Digoxin, non-DHP CCBs) and Anticoagulation (Warfarin usually preferred for valvular AF, especially mitral stenosis; assess stroke risk with CHA2DS2-VASc) (see CV005).
  • Infective Endocarditis Prophylaxis: For high-risk procedures according to guidelines (see CV002).

Monitoring

  • Monitor symptoms and functional capacity.
  • Regular clinical examination (murmurs, HF signs).
  • Regular echocardiography to assess valve function and chamber sizes.
  • Monitor INR if on Warfarin.
  • Ensure adherence to secondary prophylaxis.

Complications

  • Progressive valvular dysfunction (stenosis/regurgitation)
  • Heart Failure (decompensation)
  • Atrial Fibrillation
  • Stroke and systemic embolism (especially with AF or mitral stenosis)
  • Infective Endocarditis
  • Pulmonary Hypertension
  • Reduced quality of life
  • Premature death
  • Complications of valve surgery/intervention.

Prevention

  • Primary Prevention: Treat GAS pharyngitis effectively.
  • Secondary Prevention: Long-term antibiotic prophylaxis after ARF to prevent recurrences and development/progression of RHD.

Patient education

  • Explain RHD as damage from previous rheumatic fever.
  • Nature of the specific valve problem (stenosis/regurgitation).
  • Absolute necessity of lifelong secondary antibiotic prophylaxis (usually injections) to prevent further heart damage.
  • Need for regular cardiology follow-up and echocardiograms.
  • Symptoms of worsening disease (HF, AF, stroke) to report.
  • Importance of good dental hygiene and need for endocarditis prophylaxis before certain procedures.
  • Management of heart failure/atrial fibrillation/anticoagulation if present.
  • Pregnancy counseling (risk depends on valve lesion severity).

Uganda-specific notes

RHD is the most common cause of acquired heart disease in children and young adults in Uganda. Late presentation with severe disease and complications (HF, AF) is common. Access to echocardiography, specialist care, anticoagulation monitoring (INR), and especially cardiac surgery is severely limited. Lifelong Benzathine Penicillin prophylaxis is the most crucial intervention.

References

  • Uganda Clinical Guidelines (UCG) 2023

    Chapter 4, Section 4.1.10

  • Uganda Clinical Guidelines (UCG) 2023

    Chapter 4, Section 4.1.9 (ARF Prophylaxis details)

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