Chronic thromboembolic pulmonary hypertension: advances from bench to patient management.

O ur understanding of chronic thromboembolic pulmonary hypertension (CTEPH), in terms of its natural history, pathophysiology and management of patients, has greatly improved since the landmark descriptions given by Kenneth Moser and Nina Braunwald [1] more than 40 yrs ago. At that time, small case series were available depicting the clinical features of patients suffering from ‘‘long-standing thromboembolic pulmonary hypertension’’, ‘‘chronic massive thromboembolic obstruction’’ or ‘‘chronic obstruction of large pulmonary arteries’’. The success of a surgical procedure, namely pulmonary endarterectomy (PEA), has transformed this lethal disorder into a potentially curable form of pulmonary hypertension. As a consequence, the referral of cases suitable for surgery has gradually increased with time. At the University of San Diego (San Diego, CA, USA), more than 2,700 PEA procedures have been performed since 1970, the majority over the past 20 yrs [2–4]. European centres have subsequently contributed to broaden the knowledge on the disease [5–7].