Survey on pulmonary arterial hypertension: A picture of the Italian reality

Background. Pulmonary arterial hypertension (PAH) is a rare syndrome characterized by remodeling of the small pulmonary arterial vessels and increased pulmonary vascular resistance, which in the later stages can lead to right-sided heart failure and death. The therapeutic approach is evolving, but differences remain between countries. Methods. An Italian survey was designed to evaluate differences in the diagnosis, management and treatment of PAH patients across Italian centers. The survey was administered to 32 PAH Centers in Italy via an online questionnaire. A panel of 9 PAH experts analyzed and discussed the results. Results. Thirty Centers from 15 regions responded, thus representing the entire Italian reality. The results showed that all participating Centers perform right heart catheterization at PAH diagnosis (100%), while genetic testing is available in 73% of cases. Centers with a high patient volume have 10.4% of patients on oral monotherapy, while Centers with a low patient volume have a low percentage of patients on triple oral therapy (15%) or parenteral prostanoids (11.3%). Many Centers (70%) use parenteral prostanoids in up-front approach in incident high-risk PAH patients. Nine Centers (30%) achieve the low risk profile in more than 60% of the population followed. In line with the literature, an upfront strategy including parenteral prostanoids was associated with a high likelihood of achieving a low-risk profile. Overall, 70% of Centers have patients on the list for lung transplant. Conclusions. Italian PAH Centers have excellent adherence to the diagnostic standards recommended by European guidelines and good uniformity in therapeutic management, with some divergences related to differences in the phenotype of PAH patients. The survey also revealed a good awareness of the efficacy of parenteral prostanoids, especially if started early. Collaboration and referral of selected PAH patients to Centers with greater experience in the management of complex infusion therapies is the key to success.