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

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