Chronic Thromboembolic Pulmonary Hypertension: From Pathogenesis to the Choice of Treatment Strategy
Keywords:
chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, balloon pulmonary angioplasty, riociguat, pulmonary vascular resistance, multimodal treatmentAbstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive form of pulmonary hypertension caused by persistent organized thromboembolic obstruction and secondary remodeling of the pulmonary microcirculation. This narrative review summarizes the mechanisms that link unresolved thrombi to increased pulmonary vascular resistance and evaluates their implications for contemporary treatment selection. Evidence from clinical guidelines, expert statements, randomized trials, registries, and major observational studies was organized around pathobiology, diagnostic assessment, pulmonary endarterectomy, balloon pulmonary angioplasty, targeted pharmacotherapy, and multimodal care. The synthesis shows that operability assessment by an experienced multidisciplinary CTEPH team remains central because pulmonary endarterectomy can be curative in anatomically accessible disease. Balloon pulmonary angioplasty and riociguat provide effective options for technically inoperable or persistent/recurrent disease, while other pulmonary hypertension therapies have more limited evidence. Treatment should be individualized according to lesion distribution, hemodynamic severity, comorbidity, and local expertise. Early referral and planned multimodal management offer the best opportunity to improve symptoms, hemodynamics, and long-term outcomes.
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