Abstract

To provide an update on balloon pulmonary angioplasty (BPA) for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH), a pulmonary vascular disease that is characterized by fibro-thrombotic material mechanically obliterating major pulmonary arteries, resulting in increased pulmonary vascular resistance (PVR), progressive pulmonary hypertension (PH) combined with a microscopic pulmonary vasculopathy [1▪▪], right ventricular (RV) failure [2] and premature death. Data from a most recent CTEPH European registry (2015 and 2016) suggest significantly improved survival [3▪] of CTEPH patients compared with survival in the eighties [4], or with data from 2007 and 2009 [5]. Pulmonary endarterectomy (PEA) is still the gold-standard therapy for CTEPH [6,7]. However, only around two thirds of all CTEPH patients are amenable to surgery [3▪,5]. Patients not suitable for PEA and treated conservatively have a poor prognosis [8]. BPA may have a role for this particular group of patients. [9-11]. Currently, BPA programs are available in many countries, with excellent results at expert centers [12-15,16▪,17,18▪▪]. Based on recent data, BPA seems to have a greater impact on symptomatic and hemodynamic improvement than medical therapy with riociguat alone [15]. The evidence favoring BPA is growing, but there is still a lack of published controlled trials. In addition, treatment concepts including indication, technical performance, use of PH-targeted medication, and the concept of follow-up vary between centers. In addition, there is a significant learning curve impacting outcomes [13]. The data from the International BPA registry will provide answers for some of the open questions.

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