From the Guidelines
The average lifespan after diagnosis of pulmonary arterial hypertension (PAH) is approximately 7 years or longer with current advanced therapies. This estimate is based on recent data from registries, which show improved survival rates of 89–96% at 1 year and 73–77% at 3 years 1. The prognosis varies considerably based on PAH etiology, patient age, functional class at diagnosis, response to therapy, and presence of comorbidities.
Key Factors Influencing Survival
- PAH etiology: patients with PAH associated with portal hypertension have the worst survival, followed by PAH-CTD, IPAH, and PAH-CHD 1
- Patient age: younger patients tend to have better survival rates 1
- Functional class at diagnosis: patients with more severe symptoms at diagnosis have poorer survival rates 1
- Response to therapy: patients who respond well to therapy have better survival rates 1
- Presence of comorbidities: patients with comorbidities such as right-heart failure have poorer survival rates 1
Importance of Early Diagnosis and Treatment
Early diagnosis and prompt initiation of appropriate therapy are crucial factors in improving survival outcomes, which is why referral to specialized PAH centers is recommended for optimal management. With current advanced therapies, including endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostacyclin analogs, and soluble guanylate cyclase stimulators, median survival has improved significantly 1.
Registry Data
Registry data, such as those from the German National Register and the Giessen PH Registry, provide valuable insights into the long-term survival of patients with PAH 1. These data show that while there is an improving trend in the survival of PAH, the overall outcome still remains poor, highlighting the need for continued advances in treatment and management.
From the Research
Average Lifespan after PAH Diagnosis
The average lifespan after Pulmonary Arterial Hypertension (PAH) diagnosis can vary depending on several factors, including disease severity and treatment options.
- According to a study published in 2024 2, PAH is a rare condition with a median life expectancy of three years.
- A study published in 2022 3 reported 1-, 2-, and 3-year mortality rates of 8%, 16%, and 21%, respectively, in patients with PAH.
- Another study published in 2012 4 found that 1-, 3-, 5-, and 7-year survival rates from time of diagnosis were 85%, 68%, 57%, and 49%, respectively.
- A study published in 2021 5 found that patients with a low-risk profile had longer transplant-free survival, and survival rates differed significantly between low-, intermediate-, and high-risk groups.
Factors Affecting Lifespan
Several factors can affect the lifespan of patients with PAH, including:
- Disease severity: Patients with more severe disease tend to have poorer outcomes 3, 4, 5.
- Treatment options: The use of combination therapy and other treatment strategies can improve survival rates 3.
- Patient characteristics: Factors such as age, sex, and comorbidities can influence survival rates 3, 4, 5.
Survival Rates
Survival rates for patients with PAH can vary depending on the population being studied and the time period of the study.
- A study published in 2007 6 reported overall survival rates of 93%, 79%, 70%, 59%, 59%, and 49% at 3 months and 1,2,3,4, and 5 years, respectively, in patients with idiopathic PAH treated with inhaled iloprost.
- The study published in 2022 3 reported that mortality rates remained high in intermediate- and high-risk patients, highlighting the need for early diagnosis and aggressive treatment.