Orthodeoxia

Described for the first time in the middle of the last century, platypnea-orthodeoxia syndrome (POS) is an uncommon condition of positional dyspnea and hypoxemia, triggered by standing and relieved with recumbency. It is most commonly associated with right-to-left shunting through a patent foramen ovale (PFO) or atrial septal defect, however its pathophysiology is not entirely understood. As a rare syndrome, it remains underdiagnosed in many patients. We report two different cases that illustrate the challenge of this diagnosis and therapeutic approach...

Cardiac platypnea-orthodeoxia is a unique clinical syndrome characterized by dyspnea and deoxygenation when moving from a supine to an upright position. In this case report, we detail the experience of a 78-year-old male with persistent hypoxemia following a paradoxical embolic ischemic stroke. Despite proper management of his respiratory symptoms, the patient continued to be affected by marked dyspnea and hypoxemia, particularly when upright or in a right-sided decubitus position. Subsequent investigation revealed that his hypoxemia was a result of cardiac platypnea-orthodeoxia syndrome (POS)...

A 63-year-old man was analyzed for platypnea-orthodeoxia syndrome (POS). A complete obstruction due to bronchial carcinoma was found in the left main bronchus with bronchoscopy. After left sided pneumonectomy POS resolved completely. Historical reports suggest increased shunting through the left lung could occur in the upright position caused by decreased compression of the left pulmonary artery due to the central bronchial carcinoma. Partially absent hypoxic vasoconstriction was confirmed in this case and suggests (without a relevant shunt through a patent foramen ovale) this 'historical' hypothesis could explain the POS due to increased shunting in the upright position in this patient...

Platypnea-orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by dyspnea (platypnea) and arterial desaturation (orthodeoxia) upon changing from a lying to a standing or sitting position. The underlying physiopathological mechanisms involved are complex. Hypoxemia is attributed to the mixing of deoxygenated venous blood with arterial blood through a shunt, which can be intracardiac, or extracardiac. Patent foramen ovale is the main cause of intracardiac shunting. The transthoracic bubble contrast echocardiogram with intravenous agited saline is the most important test in the diagnosis process...

Platypnea-Orthodeoxia syndrome (POS) is a rare and poorly understood syndrome characterized by platypnea and oxygen desaturation in the upright position that is relieved by recumbency. Here, we report a case of an 84-year-old woman who had chronic hypoxia in an upright position despite using home oxygen. The patient presented for hypoxia evaluation and was noted to have a restrictive pattern on pulmonary function tests (PFT). An echocardiogram showed a prominent eustachian valve extending from inferior to superior vena cava with contrast approaching the interatrial septum...

The platypnea-orthodeoxia syndrome (POS) is a rare and often suboptimally managed condition with a complex diagnostic workup, conversely displaying an easy treatment and a good recovery of symptoms, especially if consequent to an intracardiac shunt. However, its identification is challenging, due to the several clinical manifestations, the multiple etiologies, representing often the delayed presentation of a congenital heart disease. We present a case report and review of available literature on patients with the POS secondary to a patent foramen ovale successfully treated with its closure...

Hypoxia secondary to right to left interatrial shunting in the setting of normal right-sided filling pressure is an uncommon clinical presentation. We present a case of persistent hypoxia irrespective of body position in a patient with a wide separation between the septum primum and secundum, creating a tunnel channeling flow from the right to the left atrium. Hypoxia resolved instantly following closure of the defect with an Amplatzer occluder under intracardiac echocardiography guidance. Although platypnea-orthodeoxia leads to hypoxia in the setting of normal right-sided filling pressures, the finding of a coexisting wide gap between the septum primum and secundum is likely to have worsened the hypoxia making it severe irrespective of body position...

No abstract text is available yet for this article.

Platypnea-orthodeoxia syndrome is a rare cause of positional hypoxemia and dyspnea. We present the case of a 54-year-old man with right-to-left shunting through a patent foramen ovale in the setting of metastatic cholangiocarcinoma resulting in platypnea-orthodeoxia syndrome. The shunt was originally not visualized on cardiac magnetic resonance imaging but later detected with transesophageal echocardiography. This case highlights the importance of complimentary multimodality cardiac imaging in the diagnosis of both common and uncommon disorders...

UNLABELLED: We report a rare case with a right-to-left shunt across patent foramen ovale induced by increased blood volume in the right heart structures and severe tricuspid regurgitation which was attributed to a left-to-right shunt via coronary cameral fistulas, resulting in systemic hypoxia without pulmonary hypertension. LEARNING OBJECTIVES: Right-to-left and left-to-right shunts across patent foramen ovale and coronary cameral fistulas can coexist, potentially worsened by another shunt and vice versa...

No abstract text is available yet for this article.

A 33-year-old man presented with acute dyspnoea and profound hypoxaemia, and had clubbing, greying of hair, orthodeoxia and fine inspiratory crackles. CT chest showed established pulmonary fibrosis in a usual interstitial pneumonia pattern. Additional investigations revealed a small patent foramen ovale, pancytopenia, and oesophageal varices and portal hypertensive gastropathy from liver cirrhosis. Telomere length testing demonstrated short telomeres (<1st percentile), confirming the diagnosis of a telomere biology disorder...

BACKGROUND: Patent foramen ovale (PFO) is associated with various diseases such as cryptogenic stroke, migraine, and platypnea-orthodeoxia syndrome. This study aimed to evaluate the diagnostic performance of cardiac computed tomography (CT) for PFO detection. MATERIALS AND METHODS: Consecutive patients diagnosed with atrial fibrillation and who underwent catheter ablation with pre-procedural cardiac CT and transesophageal echocardiography (TEE) were enrolled in this study...

Pulmonary arteriovenous malformations (PAVMs) are rare and most often identified in patients with hereditary hemorrhagic telangiectasia (HHT). We describe a patient with severe hypoxemia and orthodeoxia with imaging findings consistent with PAVMs. Resected lung pathologic findings confirmed the presence of numerous microscopic vascular abnormalities within the right lower lobe that was consistent with diffuse pulmonary arteriovenous shunts. Family history was negative for HHT but was positive for pulmonary arterial hypertension (PAH) in two second-degree relatives...

No abstract text is available yet for this article.

Hepatopulmonary syndrome (HPS) shows progressive dyspnea resulting from intrapulmonary atrioventricular shunts in liver cirrhosis. The comorbidity of chronic lung disease often hampers the diagnosis of progressive dyspnea in patients with HPS. Therefore, a comprehensive approach to the determination of dyspnea is required. Here, this case report shows that a patient with chronic obstructive pulmonary disease (COPD) and alcoholic liver cirrhosis was diagnosed with HPS after admission due to worsening dyspnea...

Platypnea-orthodeoxia syndrome (POS) is a rare disorder associated with coronavirus disease 2019 (COVID-19) pneumonia. However, POS may be underdiagnosed. We report the case of a 59-year-old female patient with POS complicated by pulmonary embolism in COVID-19. Imaging revealed ground-glass opacities predominantly in the lower lobes and a pulmonary embolus in the right upper lobe. She was diagnosed with POS due to marked postural discrepancies between supine and upright oxygen saturations and blood oxygenation...

We report a patient with advanced idiopathic pulmonary fibrosis (IPF), who in a single presentation experienced three complications of the disease: an acute exacerbation, spontaneous pneumomediastinum, and platypnoea-orthodeoxia syndrome. Despite there being no definitive evidence-based treatment for an acute exacerbation, we report a marked improvement with high-dose steroids. This case also highlights the importance in IPF patients of considering pneumomediastinum as a cause of non-cardiac chest pain, as well as platypnoea-orthodeoxia in those with positional dyspnoea...

No abstract text is available yet for this article.

Platypnoea orthodeoxia syndrome is a rare condition, which can be present in patients with patent foramen ovale (PFO). In this case, a 72-year-old lady presented to the emergency department with a cryptogenic stroke associated with right thalamic infarct. While in hospital, it was noticed that the patient had desaturations in the upright position, and these improved in the recumbent position, consistent with platypnoea orthodeoxia syndrome. The patient was found to have a PFO, which was then closed, and her saturations returned to normal...