Multimodal Management of Chronic Thromboembolic Pulmonary Hypertension: A Narrative Review of Treatment Selection and Sequencing

Vollständiger Abstract

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a treatable yet frequently underdiagnosed late complication of acute pulmonary embolism, in which incompletely resolved thromboemboli organise into fibrotic obstructions of the pulmonary arteries and, together with a secondary small-vessel arteriopathy affecting both obstructed and non-obstructed territories, raise pulmonary vascular resistance, impose right ventricular pressure overload, and—if left untreated—progress to right heart failure and death. Three established therapies address the disease at distinct anatomical levels: pulmonary endarterectomy, the potentially curative treatment of choice for surgically accessible disease; balloon pulmonary angioplasty, for distal inoperable lesions or residual disease; and pulmonary hypertension-targeted medical therapy, for the accompanying microvasculopathy. Their evaluation in randomised controlled trials and large registries has produced a multimodal paradigm in which these treatments are combined and sequenced according to operability and lesion distribution. This narrative review synthesises evidence published through January 2026 to accomplish the following: (i) summarise and critically appraise the contemporary evidence for each modality, distinguishing guideline-supported recommendations from observational, proof-of-concept, and expert-opinion-based practice; (ii) describe how treatments are selected, sequenced, and bridged in relation to operability and to residual or recurrent pulmonary hypertension after endarterectomy; and (iii) situate treatment within the broader chronic thromboembolic pulmonary disease spectrum and the case-finding pathway after pulmonary embolism. Rather than proposing a validated algorithm, it emphasises that, because each modality carries distinct indications and risks, decisions should be individualised through multidisciplinary assessment at expert centres, with early referral central to optimising outcomes.