Vatrano Marco, Manzi Giovanna, Picariello Claudio, D'Alto Michele, Enea Iolanda, Ghio Stefano, Caravita Sergio, Argiento Paola, Garascia Andrea, Vitulo Patrizio, Gabrielli Domenico, Agostoni Piergiuseppe, Corda Marco, Sinagra Gianfranco, Grimaldi Massimo, Scelsi Laura, Badagliacca Roberto, D'Agostino Carlo, Perrone Filardi Pasquale, Colivicchi Furio, Indolfi Ciro, Roncon Loris, Galiè Nazzareno, Oliva Fabrizio, Vizza Carmine Dario
Dipartimento di Scienze Cardiovascolari e Respiratorie, Sapienza Università di Roma, Policlinico Umberto I, Roma.
Dipartimento di Cardiologia, Ospedale Monaldi - Università degli Studi della Campania "L. Vanvitelli", Napoli.
G Ital Cardiol (Rome). 2024 Mar;25(3):192-201. doi: 10.1714/4209.42007.
Pulmonary hypertension (PH) is a frequent pathological condition worldwide, mainly secondary to cardiovascular and respiratory diseases, with a poor prognosis. Pulmonary arterial hypertension (PAH) is a rare form that affects the arterial pulmonary vasculature. PH and PAH are characterized by non-specific symptoms and a progressive increase of pulmonary vascular resistance that results in progressive, sometimes irreversible, right ventricular dysfunction. In recent years, a growing medical and social commitment on this disease allowed more accurate diagnosis in shorter times. However, the gap between guidelines and clinical practice remains a challenge for all medical doctors involved in the disease management. Considering the needs to share and describe diagnostic and therapeutic pathways, to measure the results obtained and to address the economical and organizational problems of this disease, all involved figures should collaborate to improve its prognostic impact and health expenses. In this consensus document, the PH experts of the Italian Association of Hospital Cardiologists (ANMCO) together with those of the Italian Society of Cardiology (SIC), address 1) definition, classification and unmet needs of PH and PAH; 2) classification and characteristics of centers involved in the diagnosis and treatment of the disease; 3) proposal of organization of a diagnostic-therapeutic pathway, based on robust and recent scientific evidence.
肺动脉高压（PH）是一种在全球范围内常见的病理状况，主要继发于心血管和呼吸系统疾病，预后较差。肺动脉高压（PAH）是一种影响肺动脉血管系统的罕见形式。PH和PAH的特征是非特异性症状以及肺血管阻力的逐渐增加，这会导致进行性的，有时是不可逆的右心室功能障碍。近年来，对这种疾病的医学和社会投入不断增加，使得在更短的时间内能够进行更准确的诊断。然而，指南与临床实践之间的差距仍然是所有参与该疾病管理的医生面临的挑战。考虑到需要分享和描述诊断与治疗途径、衡量所取得的结果以及解决该疾病的经济和组织问题，所有相关人员应共同合作，以改善其对预后的影响并降低医疗费用。在本共识文件中，意大利医院心脏病学家协会（ANMCO）的PH专家与意大利心脏病学会（SIC）的专家共同探讨了：1）PH和PAH的定义、分类及未满足的需求；2）参与该疾病诊断和治疗的中心的分类及特点；3）基于可靠和最新科学证据提出的诊断-治疗途径的组织建议。
