Comorbidities, Treatment and Survival Rates of Chronic Thromboembolic Pulmonary Hypertension in a Regional Centre

Abstract

Background/Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by a multitude of underlying causes, treatment modalities and prognostic outcomes. Our aim was to evaluate the underlying causes, comorbidities and survival rates of CTEPH patients. Methods: A retrospective analysis was conducted regarding the evolution of CTEPH patients confirmed by right heart catheterization under treatment with specific vasodilator medication in our centre between 2008 and 2023. Results: We treated 14 CTEPH patients, 78.57% female, 52.79 ± 13.64 years at inclusion, representing 11.29% of our pulmonary arterial hypertension registry. Initially, the distribution of patients’ NYHA class was II-14.28%, III-71.42% and IV-14.28%. In total, 71.42% of these patients were technically operable due to the central location of the thrombus, but 42.85% presented severe comorbidities and 28.57% refused the surgery or it was financially inaccessible. Only four patients were operated on by pulmonary endarterectomy (PEA). Unfortunately, all the post-PEA patients had persistent pulmonary hypertension and had to continue vasodilator treatment. Overall, 64.28% of patients had monotherapy, 21.42% double therapy and 14.28% triple therapy. Regarding underlying causes and comorbidities, we found the following incidences: 78.57% chronic venous insufficiency, 42.85% obesity, 35.71% thyroid disease, hypertension and hyperuricemia, 21.42% thrombophilia and ischemic heart disease, 14.28% atrial fibrillation, vasculitis and lung disease, and 14.28% neoplastic history and diabetes. Seven patients died (50%), six of whom were unoperated and one of whom was lost (abandoned the program). The survival rates at 1, 3, 5 and 7 years for unoperated patients were 100%, 58.3%, 29.2% and 29.2% versus 100%, 75%, 75% and 75% in post-PEA patients. Conclusions: CTEPH, marked by delayed diagnosis, multiple comorbidities and limited intervention options, requires proactive screening and comprehensive multimodal therapies, including PEA, to improve survival rates.