What is the management for a patient with heart failure class III, orthopnea, mitral valve prolapse (MVP), and moderately severe mitral regurgitation with left ventricular hypertrophy (LVH) and normal ejection fraction (EF)?

Management of Heart Failure Class III with MVP and Moderately Severe Mitral Regurgitation

This patient requires initiation of guideline-directed medical therapy (GDMT) for heart failure with preserved ejection fraction (HFpEF) as first-line management, followed by early surgical referral for mitral valve repair given symptomatic NYHA Class III status with severe structural valve disease. 1

Immediate Medical Management

Diuretics are the cornerstone of initial therapy to address the orthopnea and volume overload symptoms in this patient with NYHA Class III heart failure. 2, 3

• Loop diuretics (furosemide) should be initiated to achieve euvolemia and relieve pulmonary congestion 4
• The orthopnea indicates elevated left atrial pressure from the mitral regurgitation creating pulmonary venous congestion 2
• Volume management is critical as the regurgitant flow into the left atrium increases left atrial pressure even when LVEF remains normal 2

Beta-blockers should be initiated carefully in this patient, though with important caveats:

• Beta-blockers are recommended for heart failure management and may help reverse LV remodeling 1, 5
• However, beta-blockers may reduce forward cardiac output in severe regurgitant lesions 6
• Start at low doses and titrate cautiously while monitoring symptoms 3
• The goal is heart rate control and neurohormonal blockade without compromising cardiac output 4

Surgical Intervention Timing

Mitral valve surgery is indicated NOW, not later, because this patient meets Class I criteria for intervention. 1

The ACC/AHA guidelines are unequivocal that mitral valve surgery is recommended for symptomatic patients with chronic severe primary MR and LVEF >30%. 1 This patient has:

• NYHA Class III symptoms (orthopnea) = Stage D disease 1
• Moderately severe to severe mitral regurgitation from structural valve disease (MVP) = primary MR 1
• Normal ejection fraction = preserved systolic function 1

Critical Pitfall to Avoid

Do not wait for the ejection fraction to decline before referring for surgery. 6, 2

• In severe MR, a "normal" LVEF of 50-60% may actually represent early myocardial dysfunction because the reduced afterload from regurgitation masks true contractile impairment 2
• LVEF should ideally be >64% in severe MR; waiting for LVEF to decline results in worse surgical outcomes 6, 2
• Symptoms like dyspnea and orthopnea warrant intervention regardless of LVEF 2
• The presence of symptoms is a Class I indication for surgical intervention 2

Surgical Approach

Mitral valve repair is strongly preferred over replacement for primary MR from MVP. 1

• Repair yields superior outcomes to replacement in patients with primary MR 1
• For MVP with posterior leaflet involvement, repair is recommended with high success rates 1
• The 2023 ACC/AHA guidelines give Class I-B recommendation for MV surgery in symptomatic primary MR 1

Why Mitral Valve Replacement Alone is NOT the Answer

Mitral valve replacement should NOT be the first-line surgical approach in this patient with MVP and primary MR:

• Repair is preferred over replacement whenever anatomically feasible 1
• Replacement is reserved for cases where repair is not technically possible 1
• MVP typically involves posterior leaflet pathology that is highly amenable to repair 1

Algorithm for Management

1. Immediate (Days 1-7):

   • Initiate loop diuretics for volume overload and orthopnea 4, 2
   • Start low-dose beta-blocker with careful titration 1, 3
   • Obtain comprehensive echocardiography to confirm MR severity, assess LV dimensions (LVESD), and evaluate for pulmonary hypertension 1

2. Early (Weeks 1-4):

   • Refer to Heart Team/cardiac surgery for evaluation 1
   • Do NOT delay referral waiting for "medical optimization" in a symptomatic patient with structural valve disease 1, 2
   • Assess for other indications: pulmonary hypertension >50 mmHg, atrial fibrillation, LA enlargement 1

3. Definitive (Weeks 4-12):

   • Proceed with mitral valve repair (preferred) at a comprehensive valve center 1
   • Continue GDMT perioperatively and postoperatively 3

Special Considerations for This Case

The normal EF is reassuring but should not delay surgery in a symptomatic patient:

• The ACC/AHA guidelines recognize that patients with severe MR can be symptomatic with preserved LVEF >60% 2
• The regurgitant flow creates volume overload leading to LA enlargement and pulmonary hypertension even when LVEF remains normal 2
• Chronic severe MR imposes pure volume overload resulting in eccentric hypertrophy and LV dilation before LVEF decreases 2

The LVH finding suggests chronic hemodynamic burden:

• This indicates the heart has been compensating for the volume overload for some time 2
• Progressive LV dilation will occur if MR is left untreated 6
• Early surgical intervention prevents irreversible ventricular damage 7, 8