病例报告：一例合并阻塞性睡眠呼吸暂停的Fontan患者持续气道正压通气的管理与随访

文献信息

中文摘要

单心室心脏病患者接受分期手术姑息治疗，最终形成全腔静脉-肺动脉吻合术（Fontan手术），其循环依赖被动的肺血流。在合并阻塞性睡眠呼吸暂停（OSA）的Fontan患者中，既往报道提示持续气道正压通气（CPAP）治疗可能有害，并对该人群的心功能产生负面影响。然而，我们描述了一例成年Fontan患者，伴有心室功能障碍和重度OSA，在使用CPAP后其心脏指数显著改善。本病例突出了Fontan生理患者中可能存在的一个亚组，他们可能从CPAP治疗中获得显著的心血管获益。

英文摘要

Patients with single ventricle heart disease undergo staged surgical palliation ultimately resulting in total cavopulmonary anastomosis (Fontan procedure), with circulation that relies on passive pulmonary blood flow. In Fontan patients with comorbid obstructive sleep apnea (OSA), previous reports have suggested that continuous positive airway pressure (CPAP) therapy can be detrimental and negatively affect cardiac function in this population. However, we describe a case of an adult Fontan patient with ventricular dysfunction and severe OSA who demonstrated significant improvement his cardiac index following CPAP use. This case highlights a potential subset of patients with Fontan physiology who may derive significant cardiovascular benefit from CPAP therapy.