Front Cardiovasc Med. 2026 May 25;13:1832792. doi: 10.3389/fcvm.2026.1832792. eCollection 2026.
ABSTRACT
Chronic Thromboembolic Pulmonary Hypertension (CTEPH), classified as group 4 pulmonary hypertension (PH), is a progressive disease caused by unresolved pulmonary artery thrombi that undergo organization and fibrosis, leading to increased pulmonary vascular resistance, right heart failure, and death. Over the past decade, the understanding, diagnosis, and management of CTEPH have undergone profound transformation. This review aims to summarize and discuss recent advances in CTEPH, focusing on pathophysiological mechanisms, diagnostic innovations, therapeutic evolution, and future directions. Current evidence establishes CTEPH as a complex, multifactorial disease involving genetic susceptibility, endothelial dysfunction, inflammation, and aberrant vascular remodeling-far beyond simple mechanical obstruction. In diagnosis, novel imaging modalities including ultra-high-resolution CT, dual-energy CT, computational fluid dynamics, and artificial intelligence have significantly enhanced the sensitivity, objectivity, and functional assessment of pulmonary vascular lesions. Therapeutically, a "three-pillar" paradigm is now firmly established, with pulmonary endarterectomy (PEA) as the curative cornerstone, complemented by balloon pulmonary angioplasty (BPA) and targeted pharmacotherapy (e.g., riociguat). This paradigm is increasingly evolving toward multimodal combination strategies, including preoperative bridging therapy and management of residual PH after intervention. Despite these advances, critical challenges remain: precise identification of operable patients, optimization of surgical and interventional techniques, development of novel targeted therapies, and construction of individualized prognostic models integrating multiomics and artificial intelligence. By addressing these core issues, this review provides a comprehensive, clinically oriented perspective on the current state and future trajectory of CTEPH research and multidisciplinary management, while also discussing emerging precision medicine approaches (e.g., multi-omics and artificial intelligence) that remain investigational.
PMID:42267096 | PMC:PMC13243409 | DOI:10.3389/fcvm.2026.1832792