Castillo-Rodriguez Cristian, Soliman Dina, Juarez Michel, Roman Sherif, Abdelmalek John, Sly Zhaunn, Lahoti Ankush, Test Victor
Department of Internal Medicine Texas Tech University Health Sciences Center Lubbock Texas USA.
Division of Cardiology Texas Tech University Health Sciences Center Lubbock Texas USA.
Clin Case Rep. 2025 Aug 3;13(8):e70715. doi: 10.1002/ccr3.70715. eCollection 2025 Aug.
Platypnea-orthodeoxia syndrome is a rare disorder characterized by dyspnea (platypnea) and arterial desaturation (orthodeoxia) in the upright position, with symptom relief upon lying down. This syndrome is commonly associated with cardiac anomalies, particularly patent foramen ovale (PFO), where increased right atrial pressure facilitates right-to-left shunting, leading to hypoxemia. Other associated conditions include cirrhosis, pericardial effusion, and pneumonectomy. We present a 32-year-old female with a history of right pneumonectomy for invasive aspergillosis who presented with worsening dyspnea and a greater than 10% drop in SpO when seated compared to the supine position. Initial imaging demonstrated rightward displacement of thoracic structures, including the right atrium, but a transthoracic echocardiogram was negative for intracardiac shunt. Due to persistent hypoxemia, a transesophageal echocardiogram was performed, revealing a right-to-left shunt, an atrial septal aneurysm, and a prominent Eustachian valve. The patient underwent successful PFO closure, leading to the complete resolution of both dyspnea and hypoxemia. This case emphasizes the importance of comprehensive diagnostic evaluation in patients with POS, especially those with complex cardiopulmonary histories.
平卧呼吸-直立性低氧血症综合征是一种罕见的疾病,其特征为直立位时出现呼吸困难(平卧呼吸)和动脉血氧饱和度降低(直立性低氧血症),平卧后症状缓解。该综合征通常与心脏异常有关,尤其是卵圆孔未闭(PFO),右心房压力升高会促进右向左分流,导致低氧血症。其他相关情况包括肝硬化、心包积液和肺切除术。我们报告一名32岁女性,因侵袭性曲霉病行右肺切除术,出现呼吸困难加重,坐位时SpO₂较仰卧位下降超过10%。初始影像学检查显示胸部结构右移,包括右心房,但经胸超声心动图未发现心内分流。由于持续低氧血症,进行了经食管超声心动图检查,发现右向左分流、房间隔瘤和明显的欧氏瓣。患者成功进行了PFO封堵术,呼吸困难和低氧血症均完全缓解。该病例强调了对平卧呼吸-直立性低氧血症综合征患者进行全面诊断评估的重要性,尤其是那些有复杂心肺病史的患者。