Do individuals with pulmonary hypertension (PH) and right heart failure (RHF) survive for 15 years after diagnosis?

From the Guidelines

Individuals with pulmonary hypertension (PH) and right heart failure (RHF) are unlikely to survive for 15 years after diagnosis without modern treatment. The prognosis for these patients is generally poor, with estimated 1-year mortality rates ranging from 5% to over 10% depending on various risk factors, as outlined in the 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension 1. According to these guidelines, the presence of clinical signs of right heart failure, rapid progression of symptoms, repeated syncope, and high WHO functional class are all associated with a higher risk of mortality.

Key Factors Influencing Survival

• The underlying cause of pulmonary hypertension
• Disease severity at diagnosis
• Response to therapy
• Age
• Presence of other medical conditions
• WHO functional class
• 6-minute walking distance (6MWD)
• Cardiopulmonary exercise testing results, such as peak VO2 and VE/VCO2 slope
• Biomarker levels, including BNP and NT-proBNP
• Imaging findings, such as right atrial area and pericardial effusion

Treatment Options

Current therapies for pulmonary hypertension include medications like phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclin analogs, and soluble guanylate cyclase stimulators, which can slow disease progression and improve quality of life 1. These medications are often used in combination and can significantly improve survival prospects for patients who respond well to therapy.

Importance of Early Diagnosis and Treatment

Early diagnosis and aggressive treatment are crucial for improving long-term survival prospects for individuals with pulmonary hypertension and right heart failure. By addressing the disease early and using a combination of therapies, healthcare providers can help slow disease progression, reduce symptoms, and improve quality of life for these patients 1.

From the Research

Survival Rates for Individuals with Pulmonary Hypertension and Right Heart Failure

• The available evidence does not provide a direct answer to the question of whether individuals with pulmonary hypertension (PH) and right heart failure (RHF) can survive for 15 years after diagnosis 2, 3, 4, 5, 6.
• Studies have reported varying survival rates for patients with PH, depending on the severity of the disease and the treatment options used 3, 5.
• For example, one study reported overall survival rates of 93%, 79%, 70%, 59%, 59%, and 49% at 3 months and 1,2,3,4, and 5 years, respectively, for patients with idiopathic PAH treated with inhaled prostacyclin iloprost 3.
• Another study found that patients with PAH who had a right ventricular free wall strain worse than -12.5% had a higher risk of mortality, with a 1-year hazard ratio of 6.2 5.
• However, none of the studies provided information on long-term survival rates beyond 5 years, making it difficult to determine whether individuals with PH and RHF can survive for 15 years after diagnosis.

Factors Affecting Survival Rates

• The severity of the disease, as measured by World Health Organization functional class, is a significant predictor of survival rates 2, 3, 5.
• Treatment options, such as intravenous epoprostenol, subcutaneous treprostinil, and oral endothelin-receptor antagonists, can improve exercise capacity and reduce symptoms, but their impact on long-term survival rates is unclear 2, 3, 4.
• Right ventricular function, as measured by longitudinal systolic strain and strain rate, is an independent predictor of future right-sided heart failure, clinical deterioration, and mortality in patients with PAH 5.