What is the treatment for pulmonary hypertension with severe tricuspid regurgitation, normal Left Ventricular Ejection Fraction (LVEF) and mild left atrial dilatation?

From the Guidelines

The treatment of pulmonary hypertension with severe tricuspid regurgitation, normal left ventricular ejection fraction (LVEF), and mild left atrial dilation should prioritize a comprehensive approach targeting both the pulmonary hypertension and its cardiac consequences, with a focus on medical therapy and consideration of surgical or transcatheter valve intervention if medical therapy fails, as recommended by the most recent guidelines 1.

Initial Management

Initial management should focus on determining the underlying cause of pulmonary hypertension through right heart catheterization, as this guides specific therapy. For pulmonary arterial hypertension (Group 1), medications such as phosphodiesterase-5 inhibitors (sildenafil 20mg three times daily or tadalafil 40mg once daily), endothelin receptor antagonists (ambrisentan 5-10mg daily, bosentan 62.5-125mg twice daily, or macitentan 10mg daily), or prostacyclin analogs (epoprostenol, treprostinil, or iloprost) are recommended 1. Combination therapy is often needed for optimal results.

Management of Tricuspid Regurgitation

Diuretics (furosemide 20-80mg daily or torsemide 10-40mg daily) are essential to manage volume overload from tricuspid regurgitation, and spironolactone 25-50mg daily may provide additional benefit 1. For severe tricuspid regurgitation, surgical or transcatheter valve intervention may be considered if medical therapy fails, with consideration of the patient's underlying condition and the potential benefits and risks of intervention 1.

Monitoring and Follow-up

Regular echocardiographic monitoring is necessary to assess right ventricular function and tricuspid regurgitation severity, and to guide adjustments to medical therapy and consideration of surgical or transcatheter valve intervention 1. Oxygen supplementation should be provided to maintain saturation above 90%, and patients should be referred to a multidisciplinary team for consideration of intervention if refractory tricuspid regurgitation is present.

Key Considerations

• The severity of secondary tricuspid regurgitation may be dynamic, depending on RV function and pulmonary hypertension, and management entails focusing on underlying causes 1.
• Referral to the multidisciplinary team for consideration of intervention might be helpful in patients with refractory tricuspid regurgitation 1.
• Surgical or transcatheter valve intervention should be considered in patients with severe tricuspid regurgitation who are symptomatic or have RV dilatation, in the absence of severe RV or LV dysfunction and severe pulmonary vascular disease/hypertension 1.

From the FDA Drug Label

Sildenafil is an inhibitor of cGMP specific phosphodiesterase type-5 (PDE-5) in the smooth muscle of the pulmonary vasculature, where PDE-5 is responsible for degradation of cGMP. In patients with PAH, this can lead to vasodilation of the pulmonary vascular bed and, to a lesser degree, vasodilatation in the systemic circulation.

The treatment of pulmonary hypertension with severe tricuspid regurgitation, normal LVEF, and mild left atrial dilatation may involve the use of sildenafil 2, a phosphodiesterase type-5 inhibitor that can lead to vasodilation of the pulmonary vascular bed.

• Key points:
  • Sildenafil can help reduce pulmonary vascular resistance.
  • It is essential to monitor patients closely for potential adverse effects, such as hypotension.
  • The use of sildenafil in this specific patient population may be beneficial, but it is crucial to consider the individual patient's condition and medical history.
• Important consideration: The FDA drug label for sildenafil does not explicitly address the treatment of pulmonary hypertension with severe tricuspid regurgitation, normal LVEF, and mild left atrial dilatation. Therefore, the use of sildenafil in this context should be approached with caution and under the guidance of a qualified healthcare professional.

From the Research

Treatment of Pulmonary Hypertension with Severe Tricuspid Regurgitation

• The treatment of pulmonary hypertension with severe tricuspid regurgitation, normal left ventricular ejection fraction (LVEF), and mild left atrial dilatation is a complex issue that requires careful consideration of various factors.
• According to a study published in the European Heart Journal 3, transcatheter tricuspid valve repair (TTVR) with the MitraClip technique is a novel treatment option for patients with pulmonary hypertension and severe tricuspid regurgitation.
• The study found that TTVR was successful in reducing tricuspid regurgitation and improving symptoms in patients with pulmonary hypertension, regardless of the severity of tricuspid regurgitation.

Clinical Outcomes of Isolated Tricuspid Regurgitation

• A study published in the Journal of the American Society of Echocardiography 4 found that isolated tricuspid regurgitation is independently associated with excess mortality in patients with preserved systolic function and pulmonary hypertension.
• The study suggested that semiquantitative assessment of tricuspid regurgitation by vena contracta is an independent associate of outcome, superior to standard qualitative assessment.
• Another study published in Chest 5 found that pulmonary artery systolic pressure is a strong determinant of tricuspid regurgitation severity, but many patients with pulmonary hypertension do not exhibit significant tricuspid regurgitation.

Determinants of Tricuspid Regurgitation Severity

• The study published in Chest 5 also found that demographic characteristics, mechanical factors, remodeling of the right heart cavities, and other factors are predictive of tricuspid regurgitation severity.
• A review article published in Frontiers in Cardiovascular Medicine 6 discussed the epidemiological features and clinical burden of tricuspid regurgitation in the context of left ventricular systolic dysfunction, and highlighted the importance of considering tricuspid regurgitation as a potential therapeutic target.
• A study published in BMC Cardiovascular Disorders 7 found that significant functional tricuspid regurgitation is common in patients with atrial fibrillation and preserved left ventricular ejection fraction, and is independently associated with adverse outcomes.

Prognostic Value of Tricuspid Regurgitation

• The study published in BMC Cardiovascular Disorders 7 suggested that comprehensive echocardiographic assessment of tricuspid regurgitation is fundamental in determining the optimal management of patients with atrial fibrillation and preserved left ventricular ejection fraction.
• The study published in the European Heart Journal 3 found that the discordant diagnosis of invasive and echocardiographic pulmonary hypertension in severe tricuspid regurgitation predicts outcomes after TTVR.
• Overall, the treatment of pulmonary hypertension with severe tricuspid regurgitation, normal LVEF, and mild left atrial dilatation requires careful consideration of various factors, including the severity of tricuspid regurgitation, pulmonary artery systolic pressure, and demographic characteristics.