What is the recommended medical management for a patient with rheumatic heart disease who has developed left‑ventricular systolic dysfunction (ejection fraction <50%) and presents with NYHA class III–IV heart failure?

Medical Management of Left Ventricular Dysfunction and Heart Failure Due to Rheumatic Heart Disease

Patients with rheumatic heart disease who develop left ventricular systolic dysfunction (EF <50%) and NYHA class III–IV heart failure should receive standard guideline-directed medical therapy for heart failure with reduced ejection fraction, consisting of ACE inhibitors, beta-blockers, mineralocorticoid receptor antagonists, and diuretics, while simultaneously addressing any surgically correctable valvular lesions. 1, 2

Immediate First-Line Pharmacotherapy

ACE Inhibitor Initiation (Cornerstone Therapy)

• Start an ACE inhibitor immediately in all patients with left ventricular systolic dysfunction (EF <50%), regardless of symptom severity, unless contraindicated. 1, 2
• Begin with low doses and uptitrate every 1–2 weeks to target doses proven effective in clinical trials:
  • Enalapril 2.5 mg twice daily → 10–20 mg twice daily
  • Lisinopril 2.5–5 mg daily → 20–35 mg daily
  • Ramipril 1.25–2.5 mg daily → 5 mg twice daily 1
• Reduce or withhold diuretics for 24 hours before initiating an ACE inhibitor to prevent excessive hypotension from combined diuresis. 1
• Monitor blood pressure, serum creatinine, and potassium at 1–2 weeks after each dose increase, at 3 months, and then every 6 months. 1, 3
• If intolerable cough or angioedema develops, switch to an angiotensin-receptor blocker (valsartan or candesartan). 4, 1

Beta-Blocker Therapy (Mandatory Second Agent)

• Initiate a beta-blocker once the patient is euvolemic and stable on ACE inhibitor therapy, not during acute decompensation. 4, 1
• Use only evidence-based beta-blockers with proven mortality benefit:
  • Bisoprolol: start 1.25 mg daily → target 10 mg daily
  • Carvedilol: start 3.125 mg twice daily → target 25–50 mg twice daily
  • Metoprolol succinate: start 12.5–25 mg daily → target 200 mg daily 4, 1, 3
• Use a "start-low, go-slow" approach, uptitrating every 1–2 weeks if the preceding dose is well tolerated. 4, 1
• Absolute contraindications include asthma, severe bronchial disease, symptomatic bradycardia, or hypotension. 1

Mineralocorticoid Receptor Antagonist (Third Pillar)

• Add spironolactone 25 mg daily for NYHA class III–IV heart failure in addition to ACE inhibitor and diuretic therapy to improve survival and reduce morbidity. 4, 1, 2
• Prerequisites: serum potassium <5.0 mmol/L and estimated GFR >30 mL/min/1.73 m². 1, 3
• Monitor serum potassium and creatinine 4–6 days after initiation; if potassium rises to ≥5.5 mmol/L, reduce dose by 50% or discontinue. 1

Diuretic Management for Symptomatic Relief

• Loop diuretics are essential for rapid relief of pulmonary congestion and peripheral edema, providing immediate symptomatic improvement. 4, 1, 2
• Start furosemide 20–40 mg daily (or equivalent) and adjust based on clinical response. 1
• Diuretics must always be co-administered with an ACE inhibitor; never use as monotherapy. 1, 2
• For inadequate response, increase the loop diuretic dose or administer twice daily. 1
• For persistent fluid retention despite adequate loop diuretic dosing, combine with a thiazide diuretic (avoid thiazides if GFR <30 mL/min unless combined synergistically with a loop diuretic). 1
• In severe refractory cases, add metolazone with frequent monitoring of renal function and electrolytes. 1

Critical Monitoring Protocols

ACE Inhibitor Monitoring

• Avoid potassium-sparing diuretics during ACE inhibitor initiation to prevent life-threatening hyperkalemia. 1, 3
• Discontinue the ACE inhibitor if renal function deteriorates substantially (typically defined as creatinine increase >30% or GFR decline >25%). 1

Beta-Blocker Monitoring

• Monitor for worsening heart failure symptoms, fluid retention, hypotension, and symptomatic bradycardia during titration. 1
• If symptoms worsen, first optimize diuretic or ACE inhibitor therapy; reduce beta-blocker dose only as a secondary measure. 1
• If hypotension occurs, first reduce vasodilator dose before considering beta-blocker dose reduction. 1
• Re-introduce and uptitrate beta-blocker when the patient stabilizes. 1

Adjunctive Cardiac Glycoside Therapy

• Digoxin is indicated for atrial fibrillation with any degree of symptomatic heart failure to control ventricular rate and improve symptoms. 1
• In patients in sinus rhythm with persistent heart failure symptoms despite ACE inhibitor and diuretic therapy, digoxin may be used to improve clinical status. 1
• Typical oral dosing: 0.125–0.25 mg daily (lower dose of 0.0625–0.125 mg in older adults with normal renal function). 1

Medications to Strictly Avoid

• Non-steroidal anti-inflammatory drugs (NSAIDs) must be avoided because they worsen renal function, promote fluid retention, and antagonize ACE inhibitor efficacy, especially dangerous in patients with fluid overload. 1, 3, 2
• Calcium channel blockers (except amlodipine for specific indications) worsen heart failure outcomes and should be avoided. 2

Addressing the Underlying Valvular Pathology

• Evaluate for surgically correctable valvular lesions (mitral regurgitation, mitral stenosis, aortic regurgitation) that are driving the heart failure syndrome. 5, 6
• Mitral regurgitation and stenosis are the most common valvular lesions in rheumatic heart disease, often occurring in combination. 6
• Valve surgery is not contraindicated in acute rheumatic carditis and may be preferable to repair when severe regurgitation causes intractable left ventricular failure despite medical therapy. 5
• Two-dimensional echocardiography and Doppler studies are essential to assess valve morphology, severity of stenosis/regurgitation, and left ventricular function. 5, 6

Advanced Therapy Considerations

Cardiac Resynchronization Therapy (CRT)

• Consider CRT for patients who remain symptomatic despite first- and second-line therapy if they have:
  • Left ventricular ejection fraction <35%
  • QRS duration ≥150 ms on ECG, or
  • QRS duration 120–149 ms with mechanical dyssynchrony on echocardiography 4

Implantable Cardioverter-Defibrillator (ICD)

• Consider ICD for patients at any stage of heart failure with left ventricular systolic dysfunction if they have:
  • Sustained ventricular tachycardia, or
  • Non-sustained ventricular tachycardia inducible on electrophysiologic testing with EF <35%, or
  • QRS duration ≥120 ms with EF <30% 4

Rehabilitation and Lifestyle Modifications

• Offer a supervised group exercise-based rehabilitation program that includes psychological and educational components to all stable patients without contraindications; moderate-quality evidence shows this reduces hospital admissions and increases long-term quality of life. 4
• Encourage daily physical activity in clinically stable patients to prevent muscle deconditioning. 1
• Advise control of dietary sodium intake and restriction of excess fluid consumption in severe heart failure. 1
• Counsel patients to avoid excessive alcohol intake and to refrain from smoking. 1

Common Pitfalls to Avoid

• Do not accept initial low doses as maintenance therapy; target doses are mandatory and must be achieved through systematic uptitration every 1–2 weeks. 1, 3, 2
• Do not use potassium-sparing diuretics when initiating ACE inhibitors to prevent hyperkalemia. 1, 3
• Do not abruptly discontinue beta-blockers; abrupt withdrawal can precipitate acute decompensation. 3
• Do not use metoprolol tartrate; only metoprolol succinate has proven mortality benefit in heart failure. 3
• Do not delay beta-blocker initiation until "maximum" ACE inhibitor dose is reached; start beta-blocker once patient is stable and euvolemic on any dose of ACE inhibitor. 1