What is the management plan for rheumatic heart disease?

Management of Rheumatic Heart Disease

The comprehensive management of rheumatic heart disease (RHD) requires a multi-level approach including secondary prophylaxis with antibiotics, medical management of cardiac complications, and timely intervention for valve disease to reduce morbidity and mortality. 1

Secondary Prophylaxis

Secondary prophylaxis is the cornerstone of RHD management:

• Intramuscular benzathine penicillin G (1.2 million units every 4 weeks) is the first-line antibiotic prophylaxis regimen with the strongest evidence for preventing recurrences 1, 2
• For penicillin-allergic patients, alternatives include:
  • Oral penicillin V: 250 mg twice daily
  • Sulfadiazine: 1 g orally once daily
  • Macrolide antibiotics (for those allergic to both penicillin and sulfadiazine) 1, 2

Duration of Prophylaxis:

• For RHD with persistent valvular disease: 10 years after last attack OR until age 40, whichever is longer 1, 2
• For rheumatic fever with carditis but no residual heart disease: 10 years after last attack OR until age 21, whichever is longer 1, 2
• For rheumatic fever without carditis: 5 years after last attack OR until age 21, whichever is longer 1, 2
• Lifelong prophylaxis may be recommended for high-risk patients with significant exposure to group A streptococcus 1
• Secondary prophylaxis must continue even after valve replacement 1

Medical Management

Heart Failure Management:

• Standard guideline-directed medical therapy for LV systolic dysfunction should be used, including: 1
  • Diuretics for volume overload
  • ACE inhibitors or ARBs
  • Beta-blockers
  • Aldosterone antagonists
  • Sacubitril/valsartan when indicated
• Careful blood pressure management is essential, avoiding abrupt BP lowering in stenotic lesions 1

Atrial Fibrillation Management:

• Beta-blockers for heart rate control; digoxin may be added for additional rate control in AF 1
• Anticoagulation for stroke prevention in patients with AF 1
• Anticoagulation should also be considered for patients in sinus rhythm with severe LA dilatation, spontaneous echo contrast, or heart failure 1

Interventional Management

Percutaneous or surgical intervention is indicated for:

• Moderate-severe mitral stenosis (MVA < 1.5 cm²) with symptoms (NYHA III-IV) despite medical therapy 1
• Asymptomatic patients with severe rheumatic mitral stenosis (MVA ≤ 1.5 cm²) before pregnancy 1
• Percutaneous mitral balloon commissurotomy (PMBC) is the preferred intervention for suitable valve anatomy 1
• Valve replacement surgery when PMBC is contraindicated or unsuccessful 1

Special Considerations in Pregnancy

• Women with moderate-severe RHD should be evaluated before pregnancy and interventional therapy considered 1
• During pregnancy, patients with severe valve disease should be monitored by a dedicated heart valve team including cardiologists, surgeons, anesthesiologists, and obstetricians 1
• Medical management during pregnancy includes:
  • Beta-blockers for heart rate control
  • Diuretics for volume overload
  • Anticoagulation for women with AF or those with severe LA dilatation 1
• PMBC after the 20th week should only be performed in experienced centers 1
• Caesarean section is recommended for patients with severe mitral stenosis, severe pulmonary hypertension, or women on oral anticoagulants in pre-term labor 1

Infective Endocarditis Prophylaxis

• Antibiotic prophylaxis is reasonable before dental procedures involving manipulation of gingival tissue, periapical region of teeth, or perforation of oral mucosa in RHD patients 1
• IE prophylaxis should be given to all RHD patients unless they are already on RHD secondary prevention antibiotics 1
• For patients receiving penicillin prophylaxis who require endocarditis prophylaxis for dental procedures, an agent other than penicillin should be used 2

Additional Preventive Measures

• Optimal oral health maintenance is crucial for preventing infective endocarditis 1
• Influenza and pneumococcal vaccinations should follow standard recommendations 1
• Regular echocardiographic monitoring:
  • Every 3-5 years for mild disease
  • Every 1-2 years for moderate disease
  • Every 6-12 months for severe disease or when LV is dilating 1

Health System Approaches

• Register-based comprehensive RHD control programs are essential for tracking patients and ensuring adherence to prophylaxis 1
• Community health worker involvement can improve early diagnosis and treatment adherence 1
• Health education for patients and families about the importance of prophylaxis adherence 1
• Integration of RHD control into primary health care systems 1

Pitfalls to Avoid

• Discontinuing secondary prophylaxis too early (must follow duration guidelines based on disease severity) 1
• Inadequate anticoagulation monitoring in patients requiring warfarin 1
• Failure to recognize pregnancy as a high-risk period for women with RHD 1
• Neglecting regular follow-up echocardiography to monitor disease progression 1
• Overlooking the need for IE prophylaxis during high-risk procedures 1