Division of Pediatirc Cardiology, Congenital Heart Disease Center, Severance Cardiovascular Hospital, Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
Korean Circ J. 2015 Mar;45(2):169-73. doi: 10.4070/kcj.2015.45.2.169. Epub 2015 Mar 24.
A 29-year-old woman was referred to our institute for symptomatic hypoxemia. Her dyspnea was aggravated while sitting or standing and relieved while in supine position. She did not have any pulmonary disease. Transthoracic echocardiography and heart computed tomography revealed an underestimated small atrial septal defect (ASD) with a left-to-right shunt. A cardiac catheterization was performed to evaluate pulmonary hypertension. It revealed a normal pulmonary artery pressure and a large ASD with bidirectional shunt during Valsalva maneuver by intracardiac echocardiogram. Her arterial oxygen saturation decreased from 93% while supine to 79% while at a 15° sitting position. Thus, the patient was diagnosed with platypnea-orthodeoxia syndrome. The ASD was successfully closed with Amplatzer® (St. Jude Medical) septal occluder and both platypnea and orthodeoxia were resolved immediately after the procedure.
一位 29 岁女性因症状性低氧血症被转介至我院。她的呼吸困难在坐立或站立时加重,而在仰卧时缓解。她没有任何肺部疾病。经胸超声心动图和心脏计算机断层扫描显示,存在一个被低估的小型房间隔缺损(ASD),伴有左向右分流。进行了心导管检查以评估肺动脉高压。心内超声心动图显示,在瓦尔萨尔瓦动作时肺动脉压正常,且存在大型 ASD 双向分流。她的动脉血氧饱和度从仰卧位时的 93%下降到 15°坐姿时的 79%。因此,该患者被诊断为类立克次体-正位缺氧综合征。使用 Amplatzer®(圣犹达医疗)房间隔封堵器成功关闭 ASD,术后类立克次体和正位缺氧立即得到解决。