Chronic thromboembolic pulmonary hypertension (CTEPH): Characterization, pulmonary endarterectomy (PEA) and new therapeutic options

Abstract

Chronic thromboembolic pulmonary hypertension is considered the only type of pulmonary hypertension with a potentially curative surgical treatment. Even today it is under-diagnosed or often diagnosed late with a worsening of the prognosis. Chronic thromboembolic pulmonary hypertension presents with nonspecific symptoms and general practitioners may not be aware of the condition or potential for treatment. Current medical treatment is, at best, palliative. Pulmonary endarterectomy offers the only possibility of symptomatic and prognostic improvement, being curative in most cases in the short and long term. Not only the identification of the pathology can be difficult and delayed, moreover after the diagnosis has been established, the estimation of operability can be challenging. The operability is based on the preoperative estimation of postoperative surgical classification and probable pulmonary vascular resistance, which determine the risk of intervention and the probable outcome. This complex procedure that includes, characterization of the pathology, the surgical intervention going through the whole decision process requires a multidisciplinary collaboration of experts in pulmonary hypertension, with a dedicated surgical team, and with very precise protocols. At our center, we have built a team of dedicated specialists including radiologists, cardiologists, cardiac surgeons, anesthesiologists and physiotherapists. Together, not only we have been able to obtain surgical results comparable to higher European centers, but also, to develop and implement other therapeutic options such as pulmonary angioplasty with balloon, dedicated to patients at high risk and discarded for surgery.In the present paper, we present a review of the pathology and, as an example, our single center experience with a multidisciplinary and dedicated management of chronic thromboembolic pulmonary hypertension patients, specifically diagnosis, pulmonary endarterectomy indication, protocols and results. (C) 2017 Socialad Espanola de Cirugia Toracica-Cardiovascular. Published by Elsevier Espana, S.L.U.