Platypnea–orthodeoxia syndrome in a child: relief of symptoms after transcatheter closure of patent foramen ovale

Platypnea–orthodeoxia syndrome is a rare clinical entity characterised by positional dyspnoea and arterial desaturation while in the upright position, the symptoms generally occurring in adults. We describe a 12-year-old girl diagnosed with platypnea–orthodeoxia syndrome associated with patent foramen ovale. The symptoms resolved following percutaneous patent foramen ovale closure.

Platypnea and orthodeoxia syndrome as an uncommon clinical indication for a challenging percutaneous patent foramen ovale closure: a case report

Background Platypnea and Orthodeoxia Syndrome (POS) is a rare clinical condition characterized by positional dyspnoea and arterial desaturation. Various mechanisms are related to this syndrome. The simultaneous presence of abnormal anatomical findings [aortic root dilatation, atrial septal aneurysm (ASA), Lipomatous septum, and patent foramen ovale (PFO)] and an occurring ventilation/perfusion mismatch can modify intracardiac haemodynamics leading to POS in elderly patients. Case summary A 70-year-old man was admitted to our emergency department suffering from neurological symptoms. A brain computed tomography scan showed a subdural haematoma and the patient underwent surgical evacuation. Some days later, he experienced an acute pulmonary insufficiency (SpO2 63%) due to parenchymal basal pneumonia treated with endotracheal intubation. Two weeks later, despite pneumonia resolution, the patient’s dyspnoea became worse, experiencing deep hypoxia as soon as the patient sat up with a partial resolution on recumbent position. A transoesophageal echocardiogram with bubble-test was performed showing aortic root dilatation and a lipomatous interatrial septum characterized by the presence of tunnel-like PFO with large ASA resulting in a big right to left shunt at rest with no signs of pulmonary hypertension. The patient underwent PFO percutaneous closure intervention and a few days later O2 therapy was reduced and the patient decannulated. Discussion This case illustrates how the presence of both intracardiac and extracardiac factors may facilitate the onset of POS in aged patients. Platypnea and Orthodeoxia Syndrome should be considered in patients with unexplained dyspnoea and arterial desaturation related to orthostatism. It has a good prognosis with an improvement of quality of life if the causal factor can be treated.

Atrial flutter: yet another cause of arterial desaturation in atrial septal defect

Pulmonary hypertension is not the only cause of arterial desaturation in patients with atrial septal defect. Arterial desaturation can also occur with normal pulmonary artery pressure making it mandatory to understand the mechanism to avoid erroneous diagnosis. In this report, for the first time, we demonstrate atrial flutter as the cause of arterial desaturation in a patient with large atrial septal defect despite normal pulmonary artery pressure, which was normalised following successful radiofrequency ablation.

Platypnea–orthodeoxia syndrome after pulmonary wedge resection in a patient with severe scoliosis

Platypnea–orthodeoxia is a rare syndrome characterized by dyspnoea and arterial desaturation, exacerbated by an upright position and relieved when the subject is recumbent. We report on a unique case of a patient with severe scoliosis who presented with several episodes of arterial desaturation after right pulmonary wedge resection.

Atrial Septal Defect Presenting as Platypnea Orthodeoxia Syndrome

Platypnea orthodeoxia syndrome (POS) is an uncommon phenomenon characterized by dyspnea and arterial desaturation in an upright position improved with recumbency. It is due to an anatomical factor that allows for interatrial communication and a functional factor that promotes right to left shunting. Once identified, early intervention allows for rapid symptomatic improvement. This case highlights that among patients who present with positional hypoxia, POS should be considered as a possible etiology. Thorough evaluation is warranted to identify potentially reversible etiologies and guide management. Keywords: Platypnea orthodeoxia syndrome, Atrial septal defect, Amplatzer closure device