Diagnosis and therapy of pulmonary arterial hypertension in Italy: results of the INCIPIT2 survey

Abstract

Pulmonary arterial hypertension (PAH) is a rare clinical condition characterized by increased pulmonary vascular resistance and premature death. It is necessary to activate a pathway from the screening of pulmonary hypertension to the diagnosis of PAH, so as to use the drugs able to improve the outcome. Nowadays, in Italy, there are no data about the management of PAH in peripheral centers and about the integration of peripheral centers with those of excellence. In order to have a map of the actual Italian pathway for diagnosis and therapy of PAH, on behalf of the ANMCO Pulmonary Circulation Area, 923 Italian cardiology departments were asked to reply, on a special electronic file, to a few simple questions about their organization, from December 2012 to May 2013. 101/923 centers (48 in the North, 18 in the Middle, 35 in the South) answered correctly. 32% has no organization for PAH, 68% has a pathway for PAH diagnosis and management, and two thirds of them collaborate with excellence centers. 36 centers perform right heart catheterization with vascular reactivity (21 with nitric oxide, 8 with adenosine, 5 with epoprostenol, 2 with nitric oxide or epoprostenol). 61/101 are prescriber centers: 33 perform right heart catheterization with vascular reactivity test, 23 send their patients to the reference center for right heart catheterization, 5 perform no right heart catheterization before the prescription of specific drugs for PAH, and only 14 prescribe intravenous prostanoids. In 2011, the participating centers followed 561 patients with PAH, of whom 126 (23%) were in independent centers. With regard to the network organization of the groups, the participating centers are partly independent of the diagnostic pathway, partly refer to outside regions; in others there is a structured regional network and there are 3 Italian regions with Hub & Spoke networks that receive patients coming from other regions. Our results show the interest of Italian Cardiology to find a pathway for the diagnosis of PAH and a heterogeneity suggesting the need for a shareable pathway, thus improving the collaboration between peripheral cardiology departments and the excellence centers for PAH in a functional Hub & Spoke network.