What is the management and treatment for rheumatic heart disease?

Management and Treatment of Rheumatic Heart Disease

Secondary Antibiotic Prophylaxis: The Cornerstone of Management

Intramuscular benzathine penicillin G (1.2 million units every 4 weeks) is the first-line treatment for preventing recurrent rheumatic fever in all patients with rheumatic heart disease, with the strongest evidence for preventing disease progression. 1

Primary Prophylaxis Regimen

• Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard with Class I, Level A evidence for effectiveness 1
• For penicillin-allergic patients, use oral penicillin V 250 mg twice daily OR sulfadiazine 1 g once daily (0.5 g for patients ≤27 kg) 1
• Erythromycin is an alternative for patients with penicillin hypersensitivity, though it is considered second-line 2, 3

Duration of Prophylaxis Based on Disease Severity

The duration follows a risk-stratified approach 4, 1:

• Rheumatic fever WITH carditis AND residual valvular disease: Continue for 10 years after last episode OR until age 40, whichever is longer; consider lifelong prophylaxis for high-risk patients (teachers, daycare workers with high streptococcal exposure) 4, 1
• Rheumatic fever WITH carditis but NO residual valvular disease: Continue for 10 years after last episode OR until age 21, whichever is longer 4, 1
• Rheumatic fever WITHOUT carditis: Continue for 5 years after last episode OR until age 21, whichever is longer 4, 1

Critical Safety Consideration for Severe Disease

For patients with severe valvular disease (severe mitral stenosis, aortic stenosis, aortic insufficiency) or reduced left ventricular function, oral prophylaxis should be strongly considered instead of intramuscular benzathine penicillin G due to risk of cardiovascular compromise following injections. 5 This represents a crucial safety update based on emerging evidence of deaths following BPG injections in patients with severe rheumatic heart disease 5.

Post-Valve Surgery Prophylaxis

Secondary antibiotic prophylaxis must continue after valve surgery using the same duration guidelines as non-surgical patients—this is a critical point as valve replacement does not eliminate the risk of recurrent acute rheumatic fever. 1 Patients remain susceptible to group A streptococcus infection even after surgical intervention 1.

Medical Management of Heart Failure

For patients developing congestive heart failure secondary to rheumatic heart disease 6, 7:

• Initiate loop diuretics immediately if pulmonary congestion or peripheral edema is present 7
• Implement guideline-directed medical therapy for left ventricular systolic dysfunction: ACE inhibitors or ARBs, beta-blockers, aldosterone antagonists, and sacubitril/valsartan when indicated 6, 7
• For atrial fibrillation: Use beta-blockers or digoxin for rate control AND warfarin for anticoagulation 7
• Digoxin provides additional symptom control, particularly beneficial with concurrent atrial fibrillation 7
• If ACE inhibitors and ARBs are contraindicated, use hydralazine/isosorbide dinitrate as alternative vasodilator therapy 7

Avoid abrupt blood pressure lowering in patients with stenotic lesions as this can precipitate hemodynamic compromise 6.

Interventional Management

All patients with symptomatic severe rheumatic mitral stenosis (mitral valve area ≤1.5 cm²) should be evaluated for percutaneous mitral balloon commissurotomy (PMBC) or mitral valve surgery within 3 months of diagnosis. 6

Intervention Selection Algorithm

• PMBC is preferred when valve morphology is favorable (mobile, relatively thin leaflets free of calcium, minimal subvalvular fusion) AND mitral regurgitation is less than 2+ AND no left atrial thrombus is present 6
• Surgical intervention is indicated when valve anatomy is unfavorable, PMBC has failed, or moderate-to-severe tricuspid regurgitation requires repair 6
• Long-term outcomes show 70-80% of patients with good initial PMBC results remain symptom-free at 10 years 6

Medical therapy alone is not a substitute for definitive valve intervention in symptomatic patients with severe disease—delaying intervention increases morbidity and mortality. 6

Infective Endocarditis Prophylaxis

Current guidelines no longer recommend routine endocarditis prophylaxis for patients with rheumatic heart disease unless they have prosthetic valves or prosthetic material used in valve repair. 1

However, for patients already receiving benzathine penicillin G prophylaxis who require endocarditis prophylaxis for dental procedures 4, 3:

• Use an agent OTHER than penicillin (such as clindamycin) because oral α-hemolytic streptococci likely have developed penicillin resistance 4, 3
• Amoxicillin prophylaxis is recommended before high-risk dental or surgical procedures for patients with rheumatic heart disease, but if recently treated with penicillin/amoxicillin or with immediate penicillin hypersensitivity, use clindamycin 3

Monitoring and Surveillance

Regular echocardiographic surveillance is mandatory with frequency based on disease severity 7:

• Severe disease: Every 6-12 months 7
• Moderate disease: Every 1-2 years 7
• Mild disease: Every 3-5 years 7

Additional Preventive Measures

• Maintain optimal oral health to reduce endocarditis risk 1, 6
• Administer influenza and pneumococcal vaccinations 6, 7
• Eradicate residual Group A Streptococcus with a full therapeutic course of penicillin in acute rheumatic fever, even if throat culture is negative 1

Special Considerations for Pregnancy

Women with moderate-severe rheumatic heart disease should be evaluated before pregnancy and interventional therapy considered. 6 During pregnancy, medical management includes beta-blockers, diuretics, and anticoagulation as needed 6.

Critical Pitfalls to Avoid

• Never discontinue secondary prophylaxis too early—this is the most common error leading to recurrent rheumatic fever 6, 7
• Never assume valve replacement eliminates the need for antibiotic prophylaxis—patients remain at risk for recurrent acute rheumatic fever 1
• Never use penicillin for endocarditis prophylaxis in patients already on penicillin prophylaxis—resistance is likely 4
• Never delay valve intervention in symptomatic severe disease—medical therapy alone is inadequate 6
• Never use intramuscular benzathine penicillin G in patients with severe valvular disease or reduced ventricular function without carefully weighing risks—consider oral prophylaxis instead 5