What is the initial management for a patient with rheumatic heart disease (RHD) presenting with severe mitral stenosis (MS), severe tricuspid regurgitation (TR), right atrial (RA) and right ventricular (RV) dilatation, and severe pulmonary artery hypertension (PAH)?

Initial Management of Rheumatic Heart Disease with Severe MS, Severe TR, RA/RV Dilatation, and Severe Pulmonary Hypertension

This patient requires immediate initiation of diuretics for symptomatic relief, anticoagulation with warfarin (INR 2-3), lifelong secondary antibiotic prophylaxis with benzathine penicillin G, and urgent surgical evaluation for combined mitral and tricuspid valve surgery, as isolated medical management will not address the underlying severe valvular pathology driving the right heart failure and pulmonary hypertension. 1, 2

Immediate Medical Stabilization

Volume Management

• Loop diuretics are the cornerstone of initial therapy to relieve systemic venous congestion, hepatic congestion, and peripheral edema that characterize this presentation 3, 1
• Titrate diuretics aggressively to reduce right-sided heart failure symptoms while monitoring for hypotension and worsening renal function in this low-output state 3, 4
• Consider adding aldosterone antagonists for additional benefit, particularly given hepatic congestion which promotes secondary hyperaldosteronism 3

Anticoagulation (Mandatory)

• Initiate warfarin immediately targeting INR 2-3 for patients with rheumatic mitral stenosis and severe pulmonary hypertension, as this patient has multiple high-risk features including severely dilated left atrium and right heart chambers 3, 2, 5
• Anticoagulation is recommended when there is significantly dilated LA, even in sinus rhythm, due to high thromboembolic risk 3, 2
• Use vitamin K antagonists (warfarin), NOT NOACs, as NOACs are not validated in rheumatic valve disease 2

Secondary Prophylaxis (Critical and Often Overlooked)

• Begin benzathine penicillin G 1.2 million units IM every 4 weeks immediately to prevent recurrent acute rheumatic fever which would accelerate valve damage 2
• Continue for ≥10 years after last attack OR until age 40 (whichever is longer), and must continue even after valve surgery 2
• For penicillin allergy, use oral penicillin V, sulfadiazine, or macrolide alternatives 2

Heart Rate Control

• Beta-blockers or non-dihydropyridine calcium channel blockers for rate control, particularly important if atrial fibrillation develops 3, 2
• Digoxin is specifically recommended for rate control in atrial fibrillation with mitral stenosis 2

Definitive Surgical Strategy (Urgent Referral Required)

Combined Valve Surgery is Mandatory

• This patient requires combined mitral valve surgery AND tricuspid valve repair performed simultaneously 3, 1
• The severe TR will NOT predictably improve after isolated mitral valve surgery, especially with this degree of RA/RV dilatation and established pulmonary hypertension 3, 6
• Tricuspid valve surgery at the time of mitral surgery is a Class I recommendation and does not add appreciable operative risk 3, 1

Why Combined Surgery is Essential

• Reoperation for isolated TR after previous left-sided valve surgery carries 10-25% perioperative mortality 3
• Tricuspid annular diameter ≥3.5 cm is the best criterion for performing concomitant tricuspid annuloplasty, and this patient clearly meets criteria given severe TR with RA/RV dilatation 6
• The severe MS is driving the pulmonary hypertension which causes functional TR; however, the TR is now severe enough that it requires direct surgical correction 1, 6

Surgical Approach Details

• Tricuspid valve repair with ring annuloplasty is strongly preferred over replacement when technically feasible 3, 1
• For mitral valve: Given rheumatic etiology with severe stenosis, valve replacement is more likely than repair, though repair should be attempted if anatomy permits 3
• Consider bioprosthetic valve rather than mechanical for women of childbearing age to avoid anticoagulation hazards during pregnancy 3

Critical Preoperative Assessment

Echocardiographic Evaluation Required

• Comprehensive transthoracic echocardiography to assess mitral valve area (severe MS defined as MVA ≤1.5 cm²), severity of TR, tricuspid annular diameter, RV function, and pulmonary artery systolic pressure 3, 1
• Transesophageal echocardiography to exclude left atrial thrombus before any intervention 3, 2
• Assessment of RV function is critical as severe RV dysfunction increases surgical risk and impacts long-term outcomes 3, 1

Invasive Hemodynamic Assessment

• Right heart catheterization is useful when clinical and noninvasive data are discordant, to measure pulmonary artery pressures and pulmonary vascular resistance directly 3, 1
• This helps distinguish reversible versus irreversible pulmonary hypertension, which impacts surgical candidacy 3, 4

When Surgery May Not Be Appropriate

Contraindications to Surgery

• Conservative management only if severe irreversible RV dysfunction or irreversible pulmonary hypertension exists, where surgery is highly likely to be futile 1, 4
• The European Society of Cardiology recommends against surgery in symptomatic patients with severe secondary TR who have either severe RV dysfunction or irreversible pulmonary hypertension 1
• In such cases, transcatheter treatment may be considered at specialized heart valve centers, though evidence is limited (Class IIb) 4

Role of Percutaneous Mitral Balloon Commissurotomy (PMBC)

PMBC is NOT Appropriate Here

• PMBC is contraindicated in this patient due to severe TR, as addressing only the mitral stenosis will not resolve the established severe TR with RA/RV dilatation 3
• PMBC may be considered in selected patients with sinus rhythm, moderate atrial enlargement, and functional TR secondary to pulmonary hypertension, but this patient has progressed beyond that stage 3
• The presence of severe TR at time of presentation indicates need for combined surgical approach 1, 6

Pulmonary Hypertension Management

Medical Therapy Considerations

• Pulmonary vasodilators (such as sildenafil) might be considered in patients with severe functional TR and pulmonary hypertension who demonstrate acute responsiveness during invasive testing (Class IIb recommendation) 3, 7
• However, this is adjunctive therapy only and does not replace the need for definitive valve surgery 3
• Sildenafil increases cGMP in pulmonary vascular smooth muscle, causing vasodilation and potentially reducing RV afterload 7
• Continued medical therapy for pulmonary hypertension may be necessary after valve surgery, as pulmonary hypertension may not completely resolve despite correction of valvular lesions 1

Common Pitfalls to Avoid

Critical Errors in Management

• Do NOT delay surgical referral while attempting prolonged medical management alone—symptomatic severe MS with severe TR and pulmonary hypertension warrants immediate surgical evaluation 1, 2
• Do NOT perform isolated mitral valve surgery without addressing the severe TR—this increases risk of requiring high-risk reoperation later with worse outcomes 3, 1, 6
• Do NOT discontinue secondary antibiotic prophylaxis after valve surgery—this must continue lifelong 2
• Do NOT use NOACs instead of warfarin in rheumatic valve disease 2
• Do NOT assume TR will resolve after mitral surgery alone in a patient with this degree of RA/RV dilatation and established severe TR 6

Monitoring and Follow-up

Postoperative Surveillance

• Regular follow-up echocardiography is essential to monitor valve function, ventricular remodeling, and pulmonary pressures 1, 2
• Severe mitral stenosis typically requires monitoring every 6-12 months, though post-surgical patients require individualized surveillance protocols 2
• Continue warfarin anticoagulation postoperatively with target INR 2.5-3.5 for mechanical mitral valve or 2.0-3.0 for bioprosthetic valve 5

Alternative Consideration: Staged Approach

Two-Staged Surgery in Extreme Cases

• In rare cases of extremely severe RV dysfunction, a two-staged approach has been reported: initial mitral valve replacement followed 7 days later by tricuspid valve repair 8
• This permits progressive reduction of pulmonary pressure and partial RV remodeling before eliminating the "systolic release valve" represented by TR 8
• However, this approach is not standard and should only be considered in highly selected cases at experienced centers 8