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一例平卧呼吸-直立性低氧血症综合征病例报告:压力与血流的相互作用

A case report of platypnea-orthodeoxia syndrome: an interplay of pressure and blood flow.

作者信息

de Sousa Bispo João, Ramires Irina, Pestana Joana, Café Hugo

机构信息

Cardiology Department, Hospital de Faro, Centro Hospitalar Universitário do Algarve, Rua Leão Penedo, 8000 Faro, Portugal.

Algarve Biomedical Center, Universidade do Algarve, DCBM Ed. 2, Campus de Gambelas, 8005-319 Faro, Portugal.

出版信息

Eur Heart J Case Rep. 2019 Oct 26;3(4):1-4. doi: 10.1093/ehjcr/ytz187. eCollection 2019 Dec.

Abstract

BACKGROUND

Platypnea-orthodeoxia syndrome is a rare condition characterized by onset or worsening of dyspnoea with orthostatism (platypnea) and arterial desaturation (orthodeoxy) that is relieved by returning to a recumbent position. An acute event causing a change in the pressure relationships inside the cardiac chambers can lead to the diagnosis of a previously undiagnosed cardiac anomaly, as the following case illustrates.

CASE SUMMARY

A previously asymptomatic 80-year-old female patient was admitted in our hospital with a sudden onset ischaemic stroke. Initial evaluation, including 12-lead electrocardiogram and transthoracic echocardiogram, was unremarkable. During hospital stay, she develops pulmonary embolism, after which she complains of positional dyspnoea that develops upon sitting up, accompanied with refractory hypoxaemia that reverts on recumbent position. Transoesophageal echocardiogram revealed an interatrial septum with an exuberant hyperdynamic movement, and an abundant passage of contrast from the right atrium to the left, even without performing the Valsalva manoeuvre, compatible with an important patent foramen ovale. A percutaneous closure was performed, and patient has been symptom-free since then.

DISCUSSION

This is a case illustrates how an anomaly that has been present for 80 years can suddenly manifest itself with an array of different symptoms that can make the diagnosis challenging. A high degree of clinical suspicion is crucial for an accurate diagnosis and definitive treatment.

摘要

背景

平卧呼吸-直立性低氧血症综合征是一种罕见病症,其特征为直立位时呼吸困难发作或加重(平卧呼吸)以及动脉血氧饱和度降低(直立性低氧),而恢复卧位时症状缓解。如下例所示,导致心腔内压力关系改变的急性事件可促使诊断出先前未被诊断出的心脏异常。

病例摘要

一名80岁既往无症状的女性患者因突发缺血性卒中入住我院。包括12导联心电图和经胸超声心动图在内的初始评估未见异常。住院期间,她发生了肺栓塞,此后她主诉坐起时出现体位性呼吸困难,并伴有卧位时可缓解的难治性低氧血症。经食管超声心动图显示房间隔有活跃的高动力运动,即使未进行瓦尔萨尔瓦动作,也有大量造影剂从右心房流向左心房,这与显著的卵圆孔未闭相符。遂进行了经皮封堵,此后患者一直无症状。

讨论

本病例说明了一个存在80年的异常如何突然以一系列不同症状表现出来,从而使诊断具有挑战性。高度的临床怀疑对于准确诊断和确定性治疗至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acd2/7042127/bf02c2b0e9fe/ytz187f1.jpg

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