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骨质疏松症和严重脊柱后凸导致的平卧呼吸-直立性低氧血症:呼吸困难和低氧血症的罕见原因。

Platypnea-orthodeoxia due to osteoporosis and severe kyphosis: a rare cause for dyspnea and hypoxemia.

作者信息

Teupe Claudius H J, Groenefeld Gerian C

机构信息

Krankenhaus Sachsenhausen Teaching Hospital, Goethe-University Frankfurt, Department of Internal Medicine - Cardiology, Frankfurt;

出版信息

Heart Int. 2011;6(2):e13. doi: 10.4081/hi.2011.e13. Epub 2011 Oct 21.

Abstract

Platypnea orthodeoxia is a rare disorder characterized by dyspnea and arterial desaturation, exacerbated by the upright position and relieved when the subject is recumbent. We report the case of a 79-year old woman admitted to hospital with dyspnea who was thought to have restrictive ventilatory impairment due to osteoporosis and severe kyphosis. Interestingly, the dyspnea was aggravated in the upright position, whereas the symptoms improved in the supine position. Arterial blood gas analysis confirmed orthodeoxia. The lung function test showed only a mild obstructive and restrictive ventilation disorder. Echocardiography revealed a patent foramen ovale and an aneurysm of the atrial septum protruding into the left atrium, despite normal right atrial pressure. Transesophageal echocardiography showed a prominent Eustachian valve guiding a blood flow from the inferior vena cava directly onto the atrial septum, thereby pushing open the patent foramen ovale. Contrast-enhanced echocardiography confirmed a spontaneous right-to-left shunt through the patent foramen ovale. It was assumed that the platypnea-orthodeoxia was caused by a prominent Eustachian valve redirected to the patent foramen ovale as a result of severe osteoporosis with subsequent thoracic kyphosis and a change in the position of the entire heart. The patient underwent permanent transcatheter closure of the patent foramen ovale after hemodynamic assessment had confirmed a significant right-to-left shunt through it. After the procedure the arterial oxygen pressure increased significantly in the upright position and dyspnea improved.

摘要

直立性呼吸困难-正位低氧血症是一种罕见的疾病,其特征为呼吸困难和动脉血氧饱和度降低,直立位时症状加重,卧位时缓解。我们报告一例79岁因呼吸困难入院的女性病例,该患者因骨质疏松和严重脊柱后凸被认为存在限制性通气功能障碍。有趣的是,该患者直立位时呼吸困难加重,而仰卧位时症状改善。动脉血气分析证实存在正位低氧血症。肺功能测试仅显示轻度阻塞性和限制性通气障碍。超声心动图显示卵圆孔未闭以及房间隔瘤突入左心房,尽管右心房压力正常。经食管超声心动图显示一个明显的欧氏瓣引导下腔静脉血流直接流向房间隔,从而推开卵圆孔未闭。对比增强超声心动图证实存在经卵圆孔未闭的自发性右向左分流。推测直立性呼吸困难-正位低氧血症是由于严重骨质疏松伴胸椎后凸以及整个心脏位置改变导致欧氏瓣移位至卵圆孔未闭所致。在血流动力学评估证实存在经卵圆孔未闭的显著右向左分流后,该患者接受了卵圆孔未闭的永久性经导管封堵术。术后患者直立位时动脉血氧分压显著升高,呼吸困难改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c1b/3205784/9337ff2b4abd/hi-2011-2-e13-g001.jpg

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