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

Chronic thromboembolic pulmonary hypertension (CTEPH) is a consequence of unresolved organized thromboembolic obstruction of the pulmonary arteries, which can cause pulmonary hypertension (PH) and right heart failure (RVF). Due to its subtle signs, it is challenging to determine its exact incidence and prevalence. Furthermore, CTEPH may also present without any prior venous thromboembolic (VTE) history, contributing to underdiagnosis and undertreatment. Diagnosis requires a high degree of suspicion and is ruled out by a normal V/Q scintigraphy. Additional imaging by CT and/or conventional angiography, as well as right heart catheterization are required to confirm CTEPH and formulate treatment plans. Pulmonary thromboendarterectomy (PTE) is the treatment of choice for eligible patients and can be potentially curative. PTE has a low mortality rate of 1-2% at expert centers and offers excellent long-term survival. Furthermore, recent advances in the techniques allow distal endarterectomy with comparable outcomes. There are alternative treatment options 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 non-candidate patients, it is crucial that precapillary PH 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.