Chronic thromboembolic pulmonary hypertension and pulmonary endarterectomy: a rapid guide to diagnosis and management

D'Armini, Andrea Maria (2015) Chronic thromboembolic pulmonary hypertension and pulmonary endarterectomy: a rapid guide to diagnosis and management. Shortness of breath, 4 (1). pp. 10-13. ISSN 2281-6550

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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) represents the only type of pulmonary hypertension surgically treatable and curable, in the majority of cases, avoiding the need of lung transplant. This life-saving conservative surgery is called pulmonary endarterectomy (PEA). To date, CTEPH is still under-diagnosed, and nowadays only few physicians are aware of the benefits of PEA. Clinical suspicion is the first step for diagnosis of CTEPH. Echocardiography, lung perfusion and ventilation scan, and right heart catheterization are useful for the diagnosis. Pulmonary angiogram, CT scan, and a multidisciplinary assessment are preliminary to surgical indication for PEA. PEA may have hemodynamically dramatic and immediate effects with a prompt restoration of normal values in most cases. Riociguat, a stimulator of soluble guanylate cyclase, is the only specific drug for non-operable CTEPH patients or those with persistent/recurrent disease after surgery.

Item Type: Article
Uncontrolled Keywords: chronic tromboembolic pulmonary hypertension, CTEPH, pulmonary endarterectomy, PEA, riociguat
Subjects: 600 Tecnologia - Scienze applicate > 610 Medicina e salute (Classificare qui la tecnologia dei servizi medici)
Depositing User: Marina Spanti
Date Deposited: 19 Sep 2019 13:25
Last Modified: 19 Sep 2019 13:25
URI: http://eprints.bice.rm.cnr.it/id/eprint/12436

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