From the Guidelines
The average lifespan after diagnosis for someone with pulmonary hypertension (PH) and right heart failure (RHF) who is receiving treatment is approximately 3-5 years, with survival rates at 1,2, and 3 years being around 85%, 70%, and 63%, respectively, as demonstrated by the use of epoprostenol therapy in patients with idiopathic pulmonary arterial hypertension (IPAH) 1.
Factors Influencing Prognosis
The prognosis is heavily influenced by several factors, including:
- The underlying cause of PH
- Disease severity at diagnosis
- Response to treatment
- Presence of comorbidities
- Baseline variables such as history of right-heart failure, New York Heart Association functional class (NYHA-FC), exercise tolerance, and hemodynamics
Treatment Approach
Treatment typically involves a combination approach, including:
- Pulmonary vasodilators such as phosphodiesterase-5 inhibitors (e.g., sildenafil, tadalafil)
- Endothelin receptor antagonists (e.g., bosentan, ambrisentan)
- Prostacyclin analogs (e.g., epoprostenol, treprostinil)
- Soluble guanylate cyclase stimulators (e.g., riociguat)
- Diuretics (e.g., furosemide, spironolactone) for managing fluid overload in right heart failure
- Oxygen therapy to maintain oxygen saturation above 90%
Importance of Early Diagnosis and Treatment
Early diagnosis, prompt initiation of targeted therapy, regular cardiopulmonary assessments, and consideration for lung transplantation in eligible patients can significantly improve survival rates. The development of right heart failure specifically worsens the prognosis, as it indicates advanced disease progression where the right ventricle can no longer compensate for increased pulmonary vascular resistance, highlighting the need for aggressive management and close monitoring 1.
From the Research
Average Lifespan After Diagnosis
The average lifespan after diagnosis of someone with pulmonary hypertension (PH) and right heart failure (RHF) who is receiving treatment can vary depending on several factors, including the severity of the disease and the effectiveness of the treatment.
Treatment and Survival Rates
- With current treatments, 5-year survival has improved from 34% in 1991 to more than 60% in 2015 2.
- First-line therapy with drug combinations that target multiple biological pathways are associated with improved survival 2.
- The one-year survival from the first acute right heart failure hospitalization was 67.6%, and the two-year survival was 41.9% 3.
- Treatment with sildenafil has been shown to improve functional capacity and reduce episodes of decompensated right heart failure in patients with severe pulmonary hypertension 4, 5.
- Combination therapy with prostacyclin and sildenafil has been shown to be effective in patients with severe pulmonary hypertension 4, 5.
Factors Affecting Survival
- Higher risk of in-hospital mortality is associated with younger age, lower sodium levels, severe forms of PAH, and need of PAH combination treatment 3.
- The presence of ascites is a predictor of long-term mortality 3.
- Mortality in patients with PH and acute right heart failure remains very high, and identification of risk factors could be used as a basis for risk-adapted therapy 3.