Li Chen, Cheng Wang, Jiachen Li, Lizhi Lv, Yongtao Wu, Gang Li, Guofeng Xing, Xiaoyong Jing, Zhimin Liu, Qiang Wang
Beijing Anzhen Hospital and Capital Medical University. Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College.
China
Journal of Cardiothoracic Surgery
J Cardiothorac Surg 2026;
DOI: 10.1186/s13019-026-04085-x
Abstract
Objectives To investigate, through four clinical cases, whether compression-induced deformation of the left lower pulmonary vein by the descending aorta is an etiological factor in certain cases of severe pulmonary arterial hypertension (PAH) in children.
Study design Four pediatric patients with severe PAH and left lower pulmonary vein stenosis (PVS) due to external compression by the descending aorta underwent surgical repair under general anesthesia with hypothermic extracorporeal circulation. The first patient underwent patch augmentation of the left lower pulmonary vein using pericardium. The remaining three patients underwent left lower pulmonary vein reimplantation to relieve the compression.
Results Preoperatively, following oxygen inhalation, the total pulmonary resistance indexed to body surface area in the first three patients was 11.7, 12.6, and 20.2 Wood units × m², respectively. Intraoperative observation confirmed normally developed left lower pulmonary veins with smooth intima and no intrinsic stenosis. Postoperatively, pulmonary artery pressure normalized in all cases. Computed tomography confirmed complete relief of the venous compression. Postoperative right heart catheterization in the first three patients showed indexed total pulmonary resistances of 8.6, 9.1, and 13.9 Wood units × m². All four patients exhibited significant symptomatic improvement.
Conclusions In children with severe PAH of unexplained origin, impaired drainage of the left lower pulmonary vein secondary to compression by the descending aorta may be an initiating pathogenic factor. Surgical correction of this compression represents a novel therapeutic strategy for a subset of patients with idiopathic PAH.
Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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