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Platypnea-Orthodeoxia Syndrome Caused by an Intracardiac Shunt.

ABSTRACT: Platypnea-orthodeoxia syndrome, a rare condition characterized by posture-related dyspnea, is usually caused by an intracardiac shunt, hepatopulmonary syndrome, or shunting resulting from severe pulmonary disease. We report the case of a 33-year-old woman who presented with increasing dyspnea and oxygen desaturation when she sat up or arose. Our diagnosis was platypnea-orthodeoxia syndrome. A lead of a previously implanted pacemaker exacerbated a severe tricuspid regurgitant jet that was directed toward the patient's intra-atrial septum. Percutaneous closure of a small secundum atrial septal defect eliminated right-to-left shunting and substantially improved the patient's functional status. In addition to this case, we discuss this unusual condition.

SUBMITTER: Borgaonkar SP

PROVIDER: S-EPMC7819445 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature

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Platypnea-Orthodeoxia Syndrome Caused by an Intracardiac Shunt.

Borgaonkar Sanket P SP Lam Wilson W WW Razavi Mehdi M Parekh Dhaval R DR

Texas Heart Institute journal 20200801 4

Platypnea-orthodeoxia syndrome, a rare condition characterized by posture-related dyspnea, is usually caused by an intracardiac shunt, hepatopulmonary syndrome, or shunting resulting from severe pulmonary disease. We report the case of a 33-year-old woman who presented with increasing dyspnea and oxygen desaturation when she sat up or arose. Our diagnosis was platypnea-orthodeoxia syndrome. A lead of a previously implanted pacemaker exacerbated a severe tricuspid regurgitant jet that was directe ...[more]

PMID: 33472229

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Platypnea Orthodeoxia Syndrome Secondary to Intracardiac Shunt Following Orthotopic Liver Transplantation.

Project description:Platypnea orthodeoxia syndrome (POS) occurs when an upright position results in acute-onset hypoxemia and is relieved with recumbency. POS can be due to intracardiac shunting, intrapulmonary shunting, ventilation-perfusion mismatch, or a combination of these. We report a case of POS that developed 3 days post liver transplantation as a result of new-onset right to left shunting across a patent foramen ovale. Right heart catheterization revealed a posteriorly directed inferior vena cava likely due to altered inferior vena cava-right atrial junction anatomy as a result of liver transplantation. The patient underwent successful transcatheter patent foramen ovale closure with a 25-mm Gore Cardioform septal occluder device with immediate and sustained improvement in hypoxia.

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Platypnea-Orthodeoxia Syndrome: To Shunt or Not to Shunt, That is the Question.

Project description:Platypnea-orthodeoxia syndrome is a rare disease defined by dyspnea and deoxygenation, induced by an upright position, and relieved by recumbency. Causes include shunting through a patent foramen ovale and pulmonary arteriovenous malformations. A 79-year-old woman experienced 2 syncopal episodes at rest and presented at another hospital. In the emergency department, she was hypoxic, needing 6 L/min of oxygen. Her chest radiograph showed nothing unusual. Transthoracic echocardiograms with saline microcavitation evaluation were mildly positive early after agitated-saline administration, suggesting intracardiac shunting. She was then transferred to our center. Right-sided heart catheterization revealed no oximetric evidence of intracardiac shunting while the patient was supine and had a low right atrial pressure. However, her oxygen saturation dropped to 78% when she sat up. Repeat transthoracic echocardiography while sitting revealed a dramatically positive early saline microcavitation-uptake into the left side of the heart. Transesophageal echocardiograms showed a patent foramen ovale, with right-to-left shunting highly dependent upon body position. The patient underwent successful percutaneous patent foramen ovale closure, and her oxygen supplementation was suspended. In patients with unexplained or transient hypoxemia in which a cardiac cause is suspected, it is important to evaluate shunting in both the recumbent and upright positions. In this syndrome, elevated right atrial pressure is not necessary for significant right-to-left shunting. Percutaneous closure, if feasible, is first-line therapy in these patients.

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Diagnostic Pitfall of Platypnea-Orthodeoxia Syndrome Caused by Atrial Septal Defect after Right Pneumonectomy.

Project description:We describe a case of platypnea-orthodeoxia syndrome (POS) due to atrial septal defect (ASD) occurring in the early postoperative course of a right pneumonectomy. Deformation of the atrial septum after right pneumonectomy deviates the blood from the inferior vena cava to ASD during the sitting position creating, a massive right-to-left shunt. Diagnosis can initially be missed by making contrast bubble test through the superior vena cava. The atrial septal defect was then closed using the surgical technique, allowing an instantaneous improvement of hematosis.

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Platypnea-orthodeoxia syndrome after right lower lobectomy for lung cancer.

Project description:Platypnea-orthodeoxia syndrome (POS) is a rare condition characterized by dyspnoea and deoxygenation in an upright position that is relieved by supine positioning. There are only five published accounts of it occurring post-lobectomy. We present the case of a 72-year-old male with 3 months of supposedly unexplained dyspnoea after right lower lobectomy for lung cancer who was confirmed to have POS. We highlight the importance of recognition and management as well as provide a brief summary of the pathophysiology.

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Platypnea-Orthodeoxia Syndrome Associated with Spontaneously Ruptured Chordae Tendineae of Tricuspid Valve.

Project description:Highlights•POS is a rare clinical condition.•Symptoms are upright posture dyspnea and arterial desaturation.•Supine and sitting position oxygen saturation levels suggest right-to-left shunt.•First report of uncommon spontaneous tricuspid valve chordal rupture–induced POS.

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

Project description: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.

| S-EPMC7042127 | biostudies-literature

Platypnea-Orthodeoxia Syndrome after Thoracic Vertebral Compression Fracture Successfully Treated by Percutaneous Patent Foramen Ovale Closure.

Project description: Not available

| S-EPMC8318820 | biostudies-literature

Hypoxemia found after hospitalization with right hemiplegia due to cerebral infarction: platypnea-orthodeoxia syndrome in the older people.

Project description:An 89-year-old woman was admitted to our hospital for subacute onset of right upper and lower limb weakness and was diagnosed with acute cerebral infarction. During rehabilitation, close observation revealed that her oxygen saturation decreased in the sitting position and improved in the recumbent position without any subjective symptoms of dyspnea. Transthoracic and transesophageal echocardiography and cardiac catheterization revealed a large patent foramen ovale with an atrial septal aneurysm with right-to-left shunting through the defect, and she was diagnosed with platypnea-orthodeoxia syndrome. Her right hemiplegia caused the trunk to collapse, so the patient slumped when in sitting position, and the trunk tilted to the right forward, resulting in an increased right-to-left shunt. Her peripheral capillary oxygen saturation improved in the upright sitting position supported by therapists. This case suggests that right hemiplegia may exacerbate the symptoms of platypnea-orthodeoxia syndrome. <Learning objective: Platypnea-orthodoxia syndrome (POS) is a rare disease and often not clearly diagnosed. Previous studies have reported POS due to patent foramen ovale (PFO) after the onset of subclinical cerebral infarction. To our knowledge, this is the first case to suggest that trunk collapse during sitting due to right hemiplegia exacerbated the hypoxemia caused by POS. Moreover, 20-30% of adults may have asymptomatic PFO. POS may be detected by examining changes in oxygen saturation in different postures.>.

| S-EPMC8766348 | biostudies-literature

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

Project description:BackgroundPlatypnea 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 summaryA 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.DiscussionThis 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.

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