Hasegawa Misaki, Nagai Tomoo, Murakami Tsutomu, Ikari Yuji
Division of Cardiovascular Medicine, Department of Internal Medicine, Tokai University School of Medicine, Shimokasuya 143, Isehara-shi, Kanagawa 259-1193, Japan.
Eur Heart J Case Rep. 2020 Feb 29;4(2):1-4. doi: 10.1093/ehjcr/ytaa045. eCollection 2020 Apr.
Platypnoea-orthodeoxia syndrome (POS) is characterized by dyspnoea and arterial desaturation in the sitting position. Although its pathophysiology is complex and still needed to be investigated, the disease is one of the clinical situations which should be immediately and adequately managed by health care workers from the initial presentation.
A 66-year-old woman with a history of systemic lupus erythematosus, deep vein thrombosis, and lumbar compression fracture was admitted for evaluation of the sudden onset of dyspnoea, while in the sitting position that was relieved on placing her in the supine position. Her transoesophageal echocardiogram did reveal a deformity in the patent foramen ovale (PFO) structure with a wide gap due to aortic compression, which was markedly different from that observed in the supine position, along with massive right-to-left shunting caused by redirected venous return due to a persistent Eustachian valve. With the computed tomography and angiograms, POS was diagnosed. Then, the patient received aortic replacement and patch closure of PFO, and her symptoms were completely resolved.
Platypnoea-orthodeoxia syndrome is a condition with quite unique features and needs multiple clinical measures for the diagnosis and medical management. For all health care workers, it is essential to have a high suspicion in order to detect POS in patients with unexplained dyspnoea. Echocardiography plays a major role in establishing the diagnosis and offering the choice of therapeutic options.
平卧呼吸-直立性低氧血症综合征(POS)的特征为坐位时呼吸困难和动脉血氧饱和度降低。尽管其病理生理学复杂且仍有待研究,但该疾病是医护人员从最初就诊就应立即进行适当处理的临床情况之一。
一名66岁女性,有系统性红斑狼疮、深静脉血栓形成和腰椎压缩骨折病史,因评估突然出现的坐位呼吸困难入院,仰卧位时症状缓解。她的经食管超声心动图显示卵圆孔未闭(PFO)结构畸形,由于主动脉压迫存在宽间隙,这与仰卧位时观察到的情况明显不同,同时因持续存在的欧氏瓣导致静脉回流重定向引起大量右向左分流。通过计算机断层扫描和血管造影,诊断为POS。随后,患者接受了主动脉置换和PFO修补术,症状完全缓解。
平卧呼吸-直立性低氧血症综合征是一种具有相当独特特征的疾病,诊断和医疗管理需要多种临床措施。对于所有医护人员来说,高度怀疑对于在不明原因呼吸困难患者中检测出POS至关重要。超声心动图在确立诊断和提供治疗选择方面起主要作用。