ASPYRE-1 study: An Italian multicenter prospective study on pulmonary hypertension modality of death and validation of reveal risk score

Background: Our understanding of pulmonary arterial hypertension (PAH) pathophysiology and treatment has significantly improved over time. Within this scenario we established the Italian Network on Pulmonary Arterial Hypertension (IPHNET), promoting a national prospective registry (ASPYRE-1) to collect data on contemporary PAH patients and to define the baseline features of Italian PAH patients. The aim of our study is to show the clinical characteristics of the Italian PAH patients. This cohort of patients will be followed in the ongoing registry to provide important insight into risk stratification and mode of death. Methods: Twenty-six PAH centers were initially involved in the registry. Data were collected on anthropometric measurements, medical history, vital signs, clinical signs of heart failure, physical examination, laboratory tests (hematology and clinical chemistry), WHO functional class evaluation, non-encouraged 6-min walk test (6MWT), echocardiographic imaging and right heart catheterization (RHC) of outpatients and inpatients with PAH (Group 1) diagnosis. All the centers shared a common database for the prospective follow-up of the patients. Results: between May 2014 and January 2023, a total of 500 prevalent patients with clinical diagnosis of PAH were enrolled: idiopathic (40.6 %), heritable (4.6 %), associated with drug or toxins (0.8 %), associated with connective tissue disease (26.4 %), associated with HIV infection (4.8 %), associated with portal hypertension (3.8 %), associated with congenital heart disease (16,4 %), with features of venous/capillary involvement (1.4 %), others (1.2 %) were enrolled. According to the ERS/ESC guidelines risk assessment, 207 (41.4 %) patients were at low risk, and 286 (57.2 %) were at intermediate risk. According to the REVEAL 2.0 score, 352 (70.4 %) patients were at low risk (score < 7), 93 (18.6 %) were at intermediate risk (score 7-8), and 55 (11 %) were at high risk (score > 8), Of the total patient population, 177 (35.4 %) received monotherapy, 222 (44.4 %) received double combination therapy, and 101 (20.2 %) received triple combination therapy. Conclusion: Built as a collaborative registry of centers treating PAH patients in Italy, it holds significant promise for addressing several critical needs within the field, including patients' clinical trajectory and risk stratification.