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静息时呼吸困难:一种矛盾性平卧呼吸-直立性低氧血症综合征变体。

Breathless at rest: a paradoxical platypnea-orthodeoxia syndrome variant.

作者信息

Gussak Georg, Mehta Sandeep, Darki Amir

机构信息

Loyola University Medical Center, Maywood, Illinois, USA

Loyola University Medical Center, Maywood, Illinois, USA.

出版信息

BMJ Case Rep. 2025 Apr 29;18(4):e264421. doi: 10.1136/bcr-2024-264421.

Abstract

Platypnea-orthodeoxia syndrome (POS) is a rare and underdiagnosed clinical syndrome classically characterised by positional dyspnea (platypnea) and arterial oxygen desaturation (orthodeoxia) while upright, which improves in the supine position. There are no known cases of a POS variant where oxygenation improved during exercise and in the upright position, but worsened at rest and while supine. We present a novel case of a man in his mid-70s with symptoms and oxygen saturations paradoxical to classic POS, who was found to have a right-to-left shunt across a patent foramen ovale (PFO) with normal right atrial pressure (RAP), ascending aortic ectasia and concomitant right hemidiaphragm elevation without paralysis. On successful transcatheter PFO closure, no hypoxaemia was found in any position or exertional level. To that effect, this paradoxical variant may represent an under-recognised and underdiagnosed form of POS that warrants further investigation in patients with positional dyspnoea and hypoxaemia.

摘要

平卧呼吸-直立性低氧血症综合征(POS)是一种罕见且易被漏诊的临床综合征,其典型特征为直立时出现体位性呼吸困难(平卧呼吸)和动脉血氧饱和度降低(直立性低氧血症),而仰卧位时症状改善。目前尚无已知的POS变异病例,即运动和直立位时氧合改善,但休息和仰卧位时恶化。我们报告了一例70多岁男性的新病例,其症状和血氧饱和度与经典POS相反,发现该患者存在经未闭卵圆孔(PFO)的右向左分流,右心房压力(RAP)正常,升主动脉扩张,同时伴有右半膈肌抬高但无麻痹。成功进行经导管PFO封堵后,在任何体位或运动水平均未发现低氧血症。因此,这种矛盾的变异型可能代表一种未被充分认识和诊断的POS形式,值得对有体位性呼吸困难和低氧血症的患者进行进一步研究。

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