Case Report: Pulmonary artery biopsy findings in a patient with a BMPR2 variant-associated pulmonary arterial hypertension.

Authors

Hongxia Wu, Li Guo, Weiya Wang, Faming Jiang, Ye Wang

Published in

Frontiers in medicine. Volume 13. Pages 1848607. Epub Jul 01, 2026.

Abstract

Mutations in BMPR2 are the most common genetic cause of heritable pulmonary arterial hypertension (PAH). We report a 47-years-old male with a BMPR2 variant (c.246A > G) who presented with dyspnea and recurrent hemoptysis. Right heart catheterization confirmed PAH with a mean PAP of 46 mmHg, PAWP of 3 mmHg, and PVR of 10.75 WU. A pulmonary artery biopsy was performed to exclude alternative diagnoses such as vasculitis and chronic thromboembolism. Histology showed foam cell accumulation in the intima, infiltration of CD3⁺ T cells, CD20⁺ B cells, and CD68⁺ histiocytes in the intima and media, and mucoid deposition. These findings suggest an active inflammatory component and dysregulation of lipid metabolism in early-stage PAH. Targeted PAH therapy improved symptoms, and resolved hemoptysis after bronchial artery embolization. Although the variant is of uncertain significance, this rare biopsy provides early pathological insights into BMPR2-associated PAH. Further studies are needed to clarify mechanisms linking BMPR2, inflammation, and lipid metabolism in vascular remodeling.

PMID:
42460063
Bibliographic data and abstract were imported from PubMed on 16 Jul 2026.

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