What is the appropriate treatment and management plan for a patient with rheumatic heart disease?

Management of Rheumatic Heart Disease

Primary Treatment: Long-Term Antibiotic Prophylaxis

All patients with rheumatic heart disease require long-term secondary antibiotic prophylaxis with intramuscular benzathine penicillin G 1.2 million units every 4 weeks to prevent recurrent rheumatic fever and progressive valvular damage. 1, 2

Prophylaxis Regimens

First-line therapy:

• Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard, being approximately 10 times more effective than oral antibiotics 1
• For high-risk patients (those with residual carditis or recurrence despite adherence), administer every 3 weeks instead of every 4 weeks to maintain more consistent protective penicillin levels 3, 1, 2

Alternative regimens for penicillin allergy:

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

Duration of Prophylaxis

The duration depends on disease severity and must be strictly followed 3, 1:

• With carditis and residual heart disease: Continue for 10 years after last attack OR until age 40 years, whichever is longer 3, 1
• With carditis but no residual heart disease: Continue for 10 years after last attack OR until age 21 years, whichever is longer 3, 1
• Without carditis: Continue for 5 years after last attack OR until age 21 years, whichever is longer 3, 1
• High-risk patients with ongoing streptococcal exposure: Consider lifelong prophylaxis 1

Critical pitfall: Secondary prophylaxis must continue even after valve replacement surgery, as it prevents recurrent acute rheumatic fever from group A streptococcus infection, not valve complications 2

Acute Rheumatic Fever Management

When acute rheumatic fever is diagnosed 1:

• Administer a full therapeutic course of penicillin to eradicate residual group A Streptococcus, even if throat culture is negative 1, 2
• Initiate long-term antimicrobial prophylaxis immediately once diagnosis is confirmed 1
• Provide adjunctive therapy with acetaminophen or NSAIDs for moderate to severe symptoms or high fever, but avoid aspirin in children due to Reye's syndrome risk 1

Medical Management of Cardiac Complications

Heart Failure and Left Ventricular Dysfunction

Apply guideline-directed medical therapy when left ventricular systolic dysfunction develops 1, 2, 4:

• Diuretics for volume management 1, 2, 4
• ACE inhibitors or ARBs 1, 2, 4
• Beta-blockers 1, 2, 4
• Aldosterone antagonists 1, 2, 4
• Sacubitril/valsartan when indicated 1, 4

Critical pitfall: Avoid abrupt lowering of blood pressure in patients with stenotic valve lesions to prevent hemodynamic collapse 1, 2, 4

Atrial Fibrillation and Stroke Prevention

• Anticoagulation is required for stroke prevention in patients with atrial fibrillation 4
• Careful monitoring of anticoagulation is essential, as inadequate monitoring is a common pitfall 4

Valve Intervention Timing

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. 1, 4

Intervention Selection

Percutaneous mitral balloon commissurotomy (preferred):

• Indicated 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 4
• Long-term outcomes show 70-80% of patients with good initial PMBC results remain free of recurrent symptoms at 10 years 4

Surgical intervention:

• Indicated when valve anatomy is unfavorable, PMBC has failed, or patients have moderate-to-severe tricuspid regurgitation requiring repair 4
• Mitral valve replacement or repair is indicated for intractable hemodynamics due to severe mitral regurgitation 5

Critical pitfall: Do not delay valve intervention in symptomatic patients with severe disease, as medical therapy alone is not a substitute for definitive treatment 4

Infective Endocarditis Prophylaxis

Current guidelines do NOT recommend routine endocarditis prophylaxis for rheumatic heart disease alone. 3, 1, 2

Prophylaxis is only reasonable for patients at highest risk 3:

• Those with prosthetic cardiac valves 3, 1, 2
• Those with prosthetic material used for valve repair 3, 1, 2
• Those with previous infective endocarditis 3, 1, 2

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

Additional Preventive Measures

• Administer influenza and pneumococcal vaccinations according to standard recommendations 3, 1, 2, 4
• Maintain optimal oral health as the most important component of preventing infective endocarditis 3
• Encourage regular aerobic exercise to improve cardiovascular fitness in patients with asymptomatic valvular heart disease 3, 1, 2

Surveillance and Monitoring

Echocardiographic monitoring frequency 4:

• Mild severity: Every 3-5 years 4
• Moderate severity: Every 1-2 years 4
• Severe disease or dilating left ventricle: Every 6-12 months or more frequently 4

Critical pitfall: Neglecting regular follow-up echocardiography leads to missed opportunities for timely intervention 4

Special Considerations in Pregnancy

• Women with moderate-severe rheumatic heart disease should be evaluated before pregnancy and interventional therapy considered 4
• Medical management during pregnancy includes beta-blockers, diuretics, and anticoagulation as needed 4
• Pregnancy is a high-risk period that should not be overlooked 4