Have clinical characteristics of patients with Pulmonary Arterial Hypertension changed over the years?

Pulmonary Arterial Hypertension (PAH) is a rare and serious disease that causes a narrowing of medium and small-caliber pulmonary arteries, increasing pulmonary arterial pressure and right ventricular afterload. Population registries have been essential for better knowledge and characterization of patients with PAH, one of them being the Spanish Registry of PAH (REHAP). Patients included in REHAP between 1998 and 2021 with diagnosis of PAH were examined in order to analyze their the demographic, clinical and hemodynamic characteristics. They were stratified into 3 periods according to diagnosis date; firstly, retrospectively (1998-2007) or prospectively (2008-2021); and, secondly, within prospective cases, based on publication of ESC/ERS 2015 international guidelines (2008-2014 and 2015-2021). Clinical, exercise tolerance, hemodynamic and survival parameters were analyzed. Table 1 shows the main characteristics data. Probability of overall survival free of death and lung transplantation of patients with PAH at 1,3,5 and 10 years was 88%, 74%, 64% and 46% respectively, with a median survival of 8.5 years. Demographic, clinical and hemodynamic characteristics of patients with PAH in REHAP have changed over the years. In the last analysis period, patients were older and had more complex PAH subtypes with a worse prognosis. The 5-year survival was 64%, comparable to data from other European registries.