Pulmonary Thromboendarterectomy: The Potentially Curative Treatment of Choice for Chronic Thromboembolic Pulmonary Hypertension.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a consequence of unresolved organized thromboembolic obstruction of the pulmonary arteries, which can cause pulmonary hypertension and right ventricular failure. Owing to its subtle signs, determining its exact incidence and prevalence is challenging. Furthermore, CTEPH may also present without any prior venous thromboembolic history, contributing to underdiagnosis and undertreatment. Diagnosis requires a high degree of suspicion and is ruled out by a normal ventilation/perfusion ratio scintigraphy. Additional imaging by computed tomography and/or conventional angiography, as well as right heart catheterization, are required to confirm CTEPH and formulate treatment plans. Pulmonary thromboendarterectomy is the treatment of choice for eligible patients and can be potentially curative. Pulmonary thromboendarterectomy has a low mortality rate of 1% to 2% at expert centers and offers excellent long-term survival. Furthermore, recent advances in the techniques allow distal endarterectomy with comparable outcomes. Alternative treatment options are available for those who may not be operable or have prohibitive risks, providing some benefit. However, CTEPH is a progressive disease with low long-term survival rates if left untreated. Given excellent short- and long-term outcomes of surgery, as well as the benefits seen with other treatment modalities in noncandidate patients, it is crucial that precapillary pulmonary hypertension and CTEPH are ruled out in any patient with dyspnea of unexplained etiology. These patients should be referred to expert centers where accurate operability assessment and appropriate treatment strategies can be offered by a multidisciplinary team.