Risk factors for chronic thromboembolic pulmonary hypertension and the "Pavia Pulmonary Endarterectomy Program"

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive and lethal disease for which there is no effective medical therapy. Pulmonary endarterectomy (PEA) is therefore the treatment of choice. Perioperative mortality rates range from 5 to 24% with significant variation in the hemodynamic improvement reported. In view of the high risk of PTE, careful pre- and post-operative management is essential for a successful outcome following PEA and patients should be referred to centers with experience that provide a multidisciplinary approach. In 1994 the "Pavia Pulmonary Endarterectomy Program" was established at the Fondazione IRCCS Policlinico San Matteo - University of Pavia, Italy, in which members of a multidisciplinary team involving 11 units work in close collaboration with the aim to increase experience in the challenging problems these patients present in the evaluative, surgical, and post-operative phases of their care. Between April 1994 and August 2007, 165 PEAs have been performed in 162 patients. This review focuses on the pathogenesis and risk factors of the disease and presents insights gained from the experience of this steadily growing center.