What is the appropriate management and treatment for a patient diagnosed with Rheumatic heart disease?

Management of Rheumatic Heart Disease

Secondary Antibiotic Prophylaxis: The Cornerstone of Treatment

All patients with rheumatic heart disease must receive long-term antibiotic prophylaxis to prevent recurrent rheumatic fever and progressive valvular damage. 1

First-Line Regimen

• Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard prophylaxis regimen with the strongest evidence and approximately 10-fold greater efficacy than oral alternatives. 1, 2
• For high-risk patients (those with residual carditis or recurrence despite adherence), administer benzathine penicillin G every 3 weeks instead of every 4 weeks to maintain more consistent protective penicillin levels. 3, 1

Alternative Regimens for Penicillin Allergy

• Penicillin V 250 mg orally twice daily (or 500 mg 2-3 times daily for adolescents/adults) is the second-line option. 1
• Sulfadiazine 1 gram orally once daily for adults (0.5 gram for patients ≤27 kg) is recommended for penicillin-allergic patients. 3, 1
• Macrolide or azalide antibiotics may be used for patients allergic to both penicillin and sulfadiazine, but avoid in patients taking cytochrome P450 3A inhibitors. 1

Duration of Prophylaxis: A Risk-Stratified Approach

The duration depends on disease severity and must be individualized based on three clinical scenarios:

• Rheumatic fever WITH carditis AND residual heart disease: Continue for 10 years after last attack OR until age 40 years, whichever is longer; consider lifelong prophylaxis for high-risk patients with ongoing streptococcal exposure. 3, 1
• Rheumatic fever WITH carditis but NO residual heart disease: Continue for 10 years after last attack OR until age 21 years, whichever is longer. 1
• Rheumatic fever WITHOUT carditis: Continue for 5 years after last attack OR until age 21 years, whichever is longer. 1

Critical Implementation Details

• Before initiating long-term prophylaxis, administer a full therapeutic course of penicillin to eradicate any residual group A Streptococcus, even if throat culture is negative. 1
• Secondary prophylaxis must continue even after valve replacement surgery to eliminate the risk of recurrent acute rheumatic fever. 4

Medical Management of Cardiac Complications

Heart Failure and Left Ventricular Dysfunction

When left ventricular systolic dysfunction develops, apply guideline-directed medical therapy including:

• Diuretics for volume management 1, 2
• ACE inhibitors or ARBs for afterload reduction 1, 2
• Beta-blockers for neurohormonal blockade 1, 2
• Aldosterone antagonists when indicated 1, 2
• Sacubitril/valsartan for advanced heart failure 1

Critical pitfall: Avoid abrupt lowering of blood pressure in patients with stenotic valve lesions, as this can precipitate hemodynamic collapse. 3, 1

Valve Intervention: When Medical Management Is Not Enough

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. 2

Intervention Selection Algorithm

• PMBC is the preferred intervention 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 demonstrate that 70-80% of patients with good initial PMBC results remain free of recurrent symptoms at 10 years. 2

Infective Endocarditis Prophylaxis: Correcting a Common Misconception

Current guidelines do NOT recommend routine endocarditis prophylaxis for rheumatic heart disease alone. 1, 4 This represents a major shift from historical practice.

When Endocarditis Prophylaxis IS Indicated

Prophylaxis is reasonable only for patients at highest risk for adverse outcomes:

• Prosthetic cardiac valves 1, 4
• Prosthetic material used for valve repair 1, 4
• Previous infective endocarditis 1, 4

Important caveat: For patients already receiving penicillin for rheumatic fever prophylaxis who require endocarditis prophylaxis for dental procedures, use an agent other than penicillin. 1

Additional Preventive Measures

Vaccination and Oral Health

• Administer influenza and pneumococcal vaccinations according to standard recommendations. 1, 2
• Optimal oral health maintenance remains the most important component of preventing infective endocarditis. 3

Exercise and Lifestyle

• Encourage regular aerobic exercise to improve cardiovascular fitness in patients with asymptomatic valvular heart disease. 3, 1
• Resistive training with small free weights or repetitive isolated muscle training may be used to strengthen individual muscle groups, but avoid heavy isometric repetitive training that increases left ventricular afterload. 3

Surveillance Strategy

Echocardiographic monitoring frequency should be risk-stratified:

• Mild disease: Every 3-5 years 3
• Moderate disease: Every 1-2 years 3
• Severe disease or dilating left ventricle: Every 6-12 months 3

Common Pitfalls to Avoid

• Discontinuing secondary prophylaxis too early is the most common error leading to recurrent rheumatic fever and progressive valvular damage. 2
• Delaying valve intervention in symptomatic patients with severe disease, as medical therapy alone is not a substitute for definitive treatment. 2
• Failing to recognize pregnancy as a high-risk period requiring pre-pregnancy evaluation and potential intervention. 2
• Inadequate anticoagulation monitoring in patients with atrial fibrillation or prosthetic valves. 2