Management of Rheumatic Fever with Severe Mitral Regurgitation
For a patient with rheumatic fever and severe mitral regurgitation, initiate immediate secondary antibiotic prophylaxis with benzathine penicillin G 1.2 million units intramuscularly every 3-4 weeks, start guideline-directed medical therapy for heart failure symptoms, and evaluate urgently for surgical mitral valve repair or replacement if symptomatic or showing signs of left ventricular dysfunction. 1, 2
Immediate Medical Management
Secondary Prophylaxis (Critical First Step)
- Start benzathine penicillin G 1.2 million units intramuscularly every 4 weeks immediately to prevent recurrent rheumatic fever and further valve damage 1, 3
- Evidence suggests every 3-week dosing is superior to every 4-week dosing, with significantly fewer prophylaxis failures (0.25 vs 1.29 per 100 patient-years, p=0.015) and better resolution of mitral regurgitation (66% vs 46%, p<0.05) 4
- For penicillin-allergic patients, use oral erythromycin twice daily or sulfadiazine as alternatives 1, 3
- Continue prophylaxis for 10 years after last attack or until age 40 (whichever is longer) in patients with persistent valvular disease 1
Heart Failure Management
- Initiate guideline-directed medical therapy immediately if heart failure symptoms are present: ACE inhibitors or ARBs, beta-blockers, diuretics for volume overload, and aldosterone antagonists 1, 2
- Consider sacubitril/valsartan in appropriate patients with reduced ejection fraction 1
- Carefully manage blood pressure, avoiding abrupt lowering 1
Surgical Evaluation and Timing
Indications for Urgent Surgical Intervention
- Evaluate for mitral valve surgery within 3 months if the patient is symptomatic with severe mitral regurgitation 1
- Surgery is indicated (Class I recommendation) for symptomatic patients with severe primary mitral regurgitation regardless of left ventricular function 5, 2
- In asymptomatic patients, surgery is indicated when left ventricular ejection fraction <60% or left ventricular end-systolic diameter ≥40 mm 5, 2
- Other surgical indications include new-onset atrial fibrillation or pulmonary hypertension 2
Surgical Approach
- Mitral valve repair is strongly preferred over replacement when technically feasible, as it preserves native valve function and has better long-term outcomes 5, 2, 6
- Mitral valve replacement is typically necessary in rheumatic disease when repair is not possible due to extensive valve damage 6
- In rare cases of fulminant mitral regurgitation with ruptured chordae tendinae causing acute pulmonary edema, urgent cardiac surgery with mitral valve repair can be life-saving 7
Predictors of Need for Surgery
- Recurrent rheumatic fever (hazard ratio 7.9), tricuspid regurgitation gradient ≥42 mmHg (HR 6.3), and left ventricular end-diastolic dimension ≥6 cm (HR 8.7) are strong predictors of eventual valve surgery 8
- Patients presenting with heart failure, severe mitral regurgitation, left ventricular enlargement, and pulmonary hypertension have higher rates of requiring surgical intervention 8
Transcatheter Options (Limited Role)
- Transcatheter edge-to-edge repair (TEER) may be considered only in symptomatic patients with severe primary mitral regurgitation who are at high or prohibitive surgical risk, as determined by a heart team 5, 2
- This is a Class IIa-B recommendation with lower evidence quality compared to surgical intervention 5
Additional Prophylaxis Requirements
- Patients require additional antibiotic prophylaxis before high-risk dental procedures (those involving manipulation of gingival tissue or perforation of oral mucosa) 1, 3
- Use amoxicillin for dental prophylaxis; if recently treated with penicillin/amoxicillin or if penicillin-allergic, use clindamycin instead 3
Monitoring Protocol
- Perform echocardiography every 6-12 months for severe disease or when left ventricle is dilating 1
- Clinical evaluation every 6 months for patients with severe mitral regurgitation 2
- Monitor for progression of valve disease, development of left ventricular dysfunction, pulmonary hypertension, and atrial fibrillation 2
Critical Pitfalls to Avoid
- Never discontinue secondary prophylaxis prematurely, as recurrent rheumatic fever dramatically worsens outcomes and increases need for surgery 1, 8
- Do not delay surgical evaluation in symptomatic patients—medical therapy alone is not a substitute for definitive valve intervention 1
- Avoid inadequate anticoagulation monitoring if atrial fibrillation develops 1
- Do not overlook the need for infective endocarditis prophylaxis during high-risk procedures 1
- Recognize that approximately 25% of patients with acute rheumatic fever and carditis will ultimately require valve surgery 8