How should I manage a patient with rheumatic heart disease, including secondary prophylaxis, monitoring, and treatment of valve lesions and heart failure?

Management of Rheumatic Heart Disease

All patients with rheumatic heart disease require mandatory long-term secondary antibiotic prophylaxis with intramuscular benzathine penicillin G 1.2 million units every 4 weeks, combined with guideline-directed medical therapy for heart failure when present, regular echocardiographic surveillance, and timely valve intervention for severe symptomatic disease. 1, 2

Secondary Prophylaxis: The Cornerstone of Management

First-Line Antibiotic Regimen

• Intramuscular benzathine penicillin G 1.2 million units every 4 weeks is the gold standard for preventing recurrent rheumatic fever, with the strongest evidence for efficacy. 1, 2
• This regimen provides superior protection compared to oral alternatives due to guaranteed compliance and sustained therapeutic levels. 2

Critical Safety Consideration for High-Risk Patients

• Patients with severe mitral stenosis, aortic stenosis, aortic insufficiency, or reduced left ventricular systolic function should strongly consider oral prophylaxis instead of intramuscular benzathine penicillin G due to risk of cardiovascular compromise following injections. 3
• This represents a crucial safety update based on emerging evidence of deaths from cardiovascular collapse in severely affected patients receiving intramuscular injections. 3

Alternative Regimens for Penicillin Allergy

• Oral penicillin V 250 mg twice daily is the preferred oral alternative. 1
• Sulfadiazine 1 g once daily for patients who cannot tolerate penicillin. 1
• Macrolide antibiotics (erythromycin, clarithromycin, azithromycin) for patients allergic to both penicillin and sulfadiazine, though avoid in patients taking cytochrome P450 3A inhibitors (azole antifungals, HIV protease inhibitors, certain SSRIs). 1, 4

Duration of Prophylaxis: Risk-Stratified Approach

• Rheumatic fever with carditis and persistent valvular disease: 10 years after last attack OR until age 40 (whichever is longer)—this is the most common scenario requiring prolonged protection. 1
• Rheumatic fever with carditis but no residual valve disease: 10 years after last attack OR until age 21 (whichever is longer). 1
• Rheumatic fever without carditis: 5 years after last attack OR until age 21 (whichever is longer). 1
• Lifelong prophylaxis is recommended for patients at high risk of group A streptococcus exposure (healthcare workers, teachers, military personnel, parents of young children). 1
• Secondary prophylaxis must continue even after valve replacement surgery, as the underlying autoimmune susceptibility persists despite mechanical correction. 1, 5

Common Pitfall: Prophylaxis Adherence

• Poor adherence to benzathine penicillin G injections leaves patients vulnerable to progressive valve damage—register-based recall systems, dedicated health teams, patient education, and school-based delivery programs improve compliance. 6

Medical Management of Heart Failure

When Left Ventricular Systolic Dysfunction Develops

Standard guideline-directed medical therapy for heart failure with reduced ejection fraction should be implemented aggressively, as most patients will eventually undergo valve intervention, but medical optimization improves perioperative outcomes and quality of life. 1, 7

Diuretics: Immediate Symptomatic Relief

• Start loop diuretics immediately for pulmonary congestion or peripheral edema to provide rapid relief of dyspnea and improve exercise tolerance. 7
• If response is inadequate, increase the dose, combine loop diuretics with thiazides, or administer loop diuretics twice daily. 7
• Avoid thiazides when GFR <30 mL/min except when used synergistically with loop diuretics. 7

ACE Inhibitors: First-Line for Systolic Dysfunction

• Begin ACE inhibitors for all patients with ejection fraction <40-45%, even if asymptomatic, as they reduce mortality and prevent progressive ventricular remodeling. 7
• Start with low doses and uptitrate to target doses proven in clinical trials (not based on symptoms alone). 7
• Review and potentially reduce diuretics for 24 hours before initiating ACE inhibitors to avoid excessive hypotension. 7
• Monitor blood pressure, renal function, and electrolytes at 1-2 weeks after each dose increase, at 3 months, then every 6 months. 7
• Stop ACE inhibitors if renal function deteriorates substantially. 7
• Avoid NSAIDs as they interfere with ACE inhibitor efficacy. 7

Beta-Blockers: Add to ACE Inhibitors

• Add bisoprolol, carvedilol, or metoprolol XL/CR for all stable patients with NYHA class II-IV heart failure and reduced ejection fraction on top of ACE inhibitors and diuretics. 7

Aldosterone Antagonists: For Advanced Heart Failure

• Add spironolactone for NYHA class III-IV heart failure to improve survival and reduce hospitalizations. 7
• Avoid potassium-sparing diuretics during ACE inhibitor initiation to prevent hyperkalemia. 7

Sacubitril/Valsartan (Entresto): For Persistent Symptoms

• For patients with LVEF ≤40% who remain symptomatic (NYHA class II-III) despite at least 3 months of optimal therapy with ACE inhibitors/ARBs, beta-blockers, and mineralocorticoid receptor antagonists, switch to sacubitril/valsartan. 1, 7
• Target at least 49 mg/51 mg twice daily, with uptitration to 97 mg/103 mg twice daily every 2-4 weeks. 7
• This provides a 20% reduction in cardiovascular death or heart failure hospitalization compared to enalapril. 7

ARBs: Alternative to ACE Inhibitors

• Substitute ARBs if ACE inhibitors cause intolerable cough or angioedema. 7

Additional Heart Failure Medications

• Digoxin for symptom control, particularly beneficial if atrial fibrillation is present. 7
• Hydralazine/isosorbide dinitrate as alternative vasodilator therapy if ACE inhibitors and ARBs are contraindicated. 7

Critical Caveat for Stenotic Lesions

• Avoid abrupt lowering of blood pressure in patients with stenotic valve lesions (mitral stenosis, aortic stenosis), as they are preload-dependent and sudden afterload reduction can precipitate cardiovascular collapse. 1
• Avoid calcium channel blockers with negative inotropic effects (non-dihydropyridines) in patients with ejection fraction <50%. 7

Atrial Fibrillation Management

Rate Control and Anticoagulation

• Use beta-blockers or digoxin for rate control in patients with atrial fibrillation. 7
• Anticoagulate with warfarin for all patients with rheumatic heart disease and atrial fibrillation due to extremely high stroke risk from left atrial thrombus formation, particularly with mitral stenosis. 1, 7

Valve Intervention: Timing Is Critical

Indications for Intervention

• Evaluate all patients with symptomatic severe rheumatic mitral stenosis (mitral valve area ≤1.5 cm²) for percutaneous mitral balloon commissurotomy (PMBC) or mitral valve surgery within 3 months of diagnosis. 2
• PMBC is preferred for patients with favorable valve morphology (mobile, relatively thin leaflets free of calcium, without significant subvalvular fusion) and less than 2+ mitral regurgitation in the absence of left atrial thrombus. 2
• Surgical intervention is indicated when valve anatomy is unfavorable, PMBC has failed, or patients have moderate-to-severe tricuspid regurgitation requiring repair. 2
• Long-term outcomes show 70-80% of patients with good initial PMBC results remain free of recurrent symptoms at 10 years. 2

Critical Pitfall: Delaying Intervention

• Never delay valve intervention in symptomatic patients with severe disease—medical therapy alone is not a substitute for definitive treatment and leads to progressive irreversible myocardial damage. 2

Infective Endocarditis Prophylaxis

High-Risk Dental Procedures

• Antibiotic prophylaxis is reasonable before dental procedures involving manipulation of gingival tissue, periapical region of teeth, or perforation of oral mucosa in patients with:
  • Prosthetic cardiac valves (including transcatheter-implanted prostheses and homografts). 1
  • Prosthetic material used for valve repair (annuloplasty rings, chords, clips). 1
  • Previous infective endocarditis. 1
  • Unrepaired cyanotic congenital heart disease or repaired congenital heart disease with residual shunts or valvular regurgitation. 1
  • Cardiac transplant with valve regurgitation. 1

Nondental Procedures

• Antibiotic prophylaxis is NOT recommended for nondental procedures (TEE, esophagogastroduodenoscopy, colonoscopy, cystoscopy) in the absence of active infection, even in high-risk patients. 1

Optimal Oral Health

• Maintenance of optimal oral health remains the most important component of preventing infective endocarditis, more important than procedural prophylaxis. 1, 2

Monitoring and Surveillance

Echocardiographic Follow-Up Schedule

• Severe disease or dilating left ventricle: every 6-12 months. 2, 7
• Moderate disease: every 1-2 years. 2, 7
• Mild disease: every 3-5 years. 2, 7

Additional Preventive Measures

• Influenza and pneumococcal vaccinations should follow standard recommendations to reduce infectious complications. 1, 2
• Standard guideline-directed medical therapy for cardiac risk factors (hypertension, diabetes, hyperlipidemia) should not be neglected. 1
• Regular aerobic exercise to improve cardiovascular fitness is beneficial for most patients with asymptomatic valvular heart disease. 1

Special Considerations in Pregnancy

Preconception Evaluation

• Women with moderate-severe rheumatic heart disease should be evaluated before pregnancy and interventional therapy considered, as pregnancy dramatically increases hemodynamic stress. 2

Management During Pregnancy

• Medical management includes beta-blockers, diuretics, and anticoagulation as needed, with careful monitoring throughout pregnancy. 2

Management of Acute Sore Throat in Patients on Prophylaxis

Evaluation Approach

• Patients with sore throat and flu-like symptoms do NOT require additional antibiotics unless Group A Streptococcal infection is confirmed, as flu-like symptoms suggest viral etiology. 4
• Perform throat culture or rapid antigen detection test for Group A Streptococcus to determine if treatment beyond prophylaxis is needed. 4
• Non-GAS pharyngitis has not been described as a trigger for rheumatic fever recurrence. 4

Treatment If GAS Confirmed

• Immediate antibiotic treatment with penicillin V 250 mg orally twice daily for 10 days if GAS infection is confirmed. 4
• For penicillin-allergic patients, use macrolides (erythromycin, clarithromycin, azithromycin). 4

Critical Fact

• At least one-third of rheumatic fever cases result from asymptomatic GAS infections, and GAS infection does not have to be symptomatic to trigger recurrence—this underscores why continuous prophylaxis is essential. 4