Department of Medicine, Texas Heart Institute and Baylor-St. Luke's Medical Center; Houston, Texas 77030.
Texas Heart Institute; and Department of Cardiology, Texas Heart Institute and Baylor-St. Luke's Medical Center; Houston, Texas 77030.
Tex Heart Inst J. 2020 Aug 1;47(4):298-301. doi: 10.14503/THIJ-16-6094.
Platypnea-orthodeoxia syndrome, a rare condition characterized by posture-related dyspnea, is usually caused by an intracardiac shunt, hepatopulmonary syndrome, or shunting resulting from severe pulmonary disease. We report the case of a 33-year-old woman who presented with increasing dyspnea and oxygen desaturation when she sat up or arose. Our diagnosis was platypnea-orthodeoxia syndrome. A lead of a previously implanted pacemaker exacerbated a severe tricuspid regurgitant jet that was directed toward the patient's intra-atrial septum. Percutaneous closure of a small secundum atrial septal defect eliminated right-to-left shunting and substantially improved the patient's functional status. In addition to this case, we discuss this unusual condition.
直立性呼吸困难-低氧血症综合征是一种罕见病症,以体位相关呼吸困难为特征,通常由心内分流、肝肺综合征或严重肺部疾病引起的分流引起。我们报告了一例 33 岁女性病例,该患者在坐起或起身时呼吸困难和血氧饱和度下降加重。我们的诊断为直立性呼吸困难-低氧血症综合征。先前植入的起搏器的导联加重了严重的三尖瓣反流射流,该反流射流向患者的房间隔。经皮闭合小的房间隔缺损消除了右向左分流,并显著改善了患者的功能状态。除了这个病例,我们还讨论了这种不常见的情况。