Chronic thromboembolic pulmonary hypertension: a tribute to pulmonary endarterectomy

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Our understanding of chronic thromboembolic pulmonary hypertension (CTEPH), in terms of both its natural history and pathophysiology, has greatly improved since the landmark descriptions given by Moser and 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”. These patients shared a history of long-term dyspnoea on exertion with normal spirometry values, evidence of pulmonary hypertension, and right ventricular dysfunction in association with the presence of marked defects at the ventilation/perfusion scan; the occurrence of a single or multiple venous thromboembolism episodes, usually several years previously, was constantly mentioned in the diagnostic work-up 2–5. The success of a surgical procedure, namely pulmonary endarterectomy, has transformed this lethal disorder into a potentially curable form of pulmonary hypertension 1. 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) there were 41 operated cases in 1980, rising to 323 cases in 1992 6, 7. This has enabled us to broaden our knowledge, with the help of further involvement from European centres 8–11]. Forty years ago, the frequency of CTEPH was estimated to be 0.1% of patients who survived an episode of pulmonary embolism 12 …