Cardiology Division, University Hospital, Geneva, Switzerland.
Eur J Intern Med. 2013 Dec;24(8):813-7. doi: 10.1016/j.ejim.2013.08.698. Epub 2013 Sep 3.
Platypnea-orthodeoxia syndrome (POS) is a rare clinical phenomenon, associating normal oxygen saturation in a supine position and arterial hypoxemia in an upright position. This pathology can be secondary to an intracardiac shunt, a pulmonary vascular shunt or a ventilation-perfusion mismatch. Cardiac POS occurs in the presence of a right-to-left cardiac shunt, most commonly through a patent foramen ovale (PFO).
From our single-center prospective database of percutaneous PFO closure we identified five patients (4 females, mean age: 77 ± 11 years) out of 224 (2.2%) patients with a PFO who presented with a POS of cardiac origin. Transthoracic and transoesophageal echocardiographic examinations revealed the underlying mechanisms of POS and the diagnosis was confirmed by right-and-left cardiac catheterization (RLC) and by measuring serial blood oxygen saturation in the pulmonary veins and left atrium in supine and upright positions. PFO was associated with atrial septal aneurysm and a persistent prominent Eustachian valve in 3 patients. All patients underwent a successful percutaneous PFO closure without any immediate or subsequent complications (mean follow-up of 24 ± 18 months). Immediately after the procedure, mean arterial oxygen saturation improved from 83% ± 3 to 93% ± 2 in an upright position and symptoms disappeared.
POS is a rare and under-diagnosed pathology that must be actively investigated in the presence of position-dependent hypoxemia. The diagnostic work-up must exclude other causes of hypoxemia and confirm the intracardiac shunt using either contrast echocardiography or RLC. For cardiac POS, percutaneous PFO closure is a safe and effective technique that immediately relieves orthodeoxia and patient symptoms.
直立性低氧血症-通气过度综合征(POS)是一种罕见的临床现象,其特征为仰卧位时氧饱和度正常,而直立位时出现动脉低氧血症。这种病理变化可能继发于心内分流、肺血管分流或通气-灌注不匹配。心脏 POS 发生于存在右向左心内分流的情况下,最常见的原因为卵圆孔未闭(PFO)。
在我们的经皮 PFO 封堵单中心前瞻性数据库中,我们从 224 例(2.2%)PFO 患者中发现了 5 例(4 例女性,平均年龄 77 ± 11 岁)存在源于心脏的 POS。经胸和经食管超声心动图检查揭示了 POS 的潜在机制,通过右心和左心导管检查(RLC)以及测量仰卧位和直立位时肺静脉和左心房的连续血氧饱和度来确诊。3 例患者的 PFO 与房间隔瘤和持续突出的咽鼓管有关。所有患者均成功接受了经皮 PFO 封堵术,无任何即刻或随后的并发症(平均随访 24 ± 18 个月)。在手术完成后,患者直立位时的平均动脉血氧饱和度从 83%±3%改善至 93%±2%,症状消失。
POS 是一种罕见且诊断不足的疾病,在存在体位性低氧血症时必须积极进行检查。诊断性检查必须排除其他低氧血症的原因,并通过对比超声心动图或 RLC 证实心内分流。对于心脏 POS,经皮 PFO 封堵术是一种安全有效的技术,可立即缓解直立性低氧血症和患者的症状。