What is the management plan for a patient with severe Mitral Regurgitation (MR) and severe Tricuspid Regurgitation (TR) with severe Pulmonary Arterial Hypertension (PAH)?

Management of Severe Mitral Regurgitation, Severe Tricuspid Regurgitation with Severe Pulmonary Arterial Hypertension

Surgical intervention with combined mitral valve repair/replacement and tricuspid valve repair is the recommended treatment for patients with severe mitral regurgitation (MR) and severe tricuspid regurgitation (TR) with severe pulmonary arterial hypertension (PAH). 1

Initial Medical Management

• Diuretics are essential for managing volume overload and reducing right-sided heart failure symptoms in patients with severe TR and PAH 1
• Medical therapies to reduce pulmonary artery pressures should be considered in patients with severe functional TR and PAH before and after surgical intervention 1
• Pulmonary vasodilators such as sildenafil (phosphodiesterase-5 inhibitor) may be beneficial in reducing pulmonary vascular resistance, improving exercise capacity, and decreasing TR severity in patients with PAH 2, 3, 4
• Treprostinil injection is indicated for PAH to diminish symptoms associated with exercise in patients with NYHA Functional Class II-IV symptoms 5

Surgical Decision-Making

• Tricuspid valve surgery is strongly recommended for patients with severe TR who are undergoing left-sided valve surgery (Class I recommendation) 1
• Mitral valve repair is preferred over mitral valve replacement when technically feasible, particularly for primary MR involving the posterior leaflet 1, 6
• For patients with severe secondary MR and TR undergoing surgery, concomitant repair of both valves should be performed 1
• The presence of severe PAH increases surgical risk but is not an absolute contraindication to surgery if the PAH is potentially reversible after correction of the valvular lesions 1

Preoperative Assessment

• Comprehensive evaluation with transthoracic echocardiography (TTE) is essential to assess valve anatomy, severity of regurgitation, chamber sizes, and estimate pulmonary artery pressure 1
• Invasive measurement of pulmonary artery pressures and pulmonary vascular resistance is useful when clinical and noninvasive data are discordant 1
• Assessment of right ventricular function is critical, as severe RV dysfunction may increase surgical risk and impact long-term outcomes 1, 7
• Cardiac magnetic resonance imaging (CMR) may provide more accurate assessment of RV function and TR quantification in patients with suboptimal echocardiographic images 1, 8

Specific Surgical Considerations

• For severe MR, mitral valve repair is recommended for symptomatic patients with LVEF >30% (Class I) 1
• For severe TR, tricuspid valve repair with annuloplasty is preferred over replacement when feasible 1
• Patients with severe TR and signs of right-sided heart failure should undergo tricuspid valve surgery even if the PAH is severe, as TR may not improve after treatment of left-sided valve disease alone 1
• The choice of prosthetic valve (mechanical vs. bioprosthetic) should consider the patient's age, need for anticoagulation, and risk of reoperation 1

Postoperative Management

• Continued medical therapy for PAH is often necessary after valve surgery, as PAH may not completely resolve despite correction of valvular lesions 1, 6
• Sildenafil has been shown to improve exercise capacity, hemodynamic measures, and functional class in patients with PAH, with benefits starting as early as 2 weeks of treatment 3, 4
• Higher doses of sildenafil (up to 150-225 mg/day) may provide additional benefits in severe PAH cases, though standard dosing is 20 mg three times daily 4, 9
• Regular follow-up with echocardiography is essential to monitor valve function, ventricular remodeling, and pulmonary pressures 6

Prognostic Considerations

• Severe TR is strongly predictive of increased 5-year mortality risk in PAH patients, even after adjustment for other risk factors 7
• The presence of both severe MR and severe TR with PAH indicates advanced disease with worse prognosis if left untreated 6, 7
• TR severity correlates with PAH severity, with greater TR associated with higher right atrial pressure, lower cardiac index, and worse functional capacity 7, 8
• Early intervention before the development of irreversible RV dysfunction may improve long-term outcomes 1

Special Considerations

• In patients with prohibitively high surgical risk, transcatheter options might be considered for MR, though the presence of both MR and TR with PAH may limit these options 6
• Reoperation for isolated TR after previous left-sided valve surgery carries higher risk (10-25% mortality) and should be carefully considered in patients with severe PAH 1
• Caution should be exercised when considering surgery in patients with severe RV systolic dysfunction or irreversible pulmonary hypertension due to the risk of RV failure after operation 1