2013
DOI: 10.2169/internalmedicine.52.0578
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Platypnea-orthodeoxia Syndrome Caused by a Latent Atrial Septal Defect

Abstract: A 79-year-old woman presented with unexplained hypoxia that became exacerbated by an upright posture (platypnea-orthodeoxia syndrome). A 99m Tc-macroaggregated albumin pulmonary perfusion scan revealed a right to left shunt of 25.5% in the supine position and 32.3% in the sitting position. A dynamic CT scan and a transoesophageal echocardiogram confirmed the presence of a shunt across an atrial septal defect (ASD). A percutaneous transcatheter closure of the defect significantly improved the patient's blood ox… Show more

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Cited by 9 publications
(4 citation statements)
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“…By 2012, only 188 patients had been described with this syndrome, 1 making the diagnosis challenging and time-consuming. Although the pathophysiology of this entity remains somewhat uncertain, 2 there are basically three recognized etiologies: cardiac conditions with intracardiac shunting, pulmonary diseases with ventilation/perfusion mismatch and hepatic entities with pulmonary arteriovenous shunts. 1,3 Among these potential causes, cardiac conditions are the most frequently associated with this syndrome, 4 with patent foramen ovale (PFO) being the most common cause of intracardiac shunting, followed by atrial septal defects and atrial septal aneurysms.…”
Section: Introductionmentioning
confidence: 99%
“…By 2012, only 188 patients had been described with this syndrome, 1 making the diagnosis challenging and time-consuming. Although the pathophysiology of this entity remains somewhat uncertain, 2 there are basically three recognized etiologies: cardiac conditions with intracardiac shunting, pulmonary diseases with ventilation/perfusion mismatch and hepatic entities with pulmonary arteriovenous shunts. 1,3 Among these potential causes, cardiac conditions are the most frequently associated with this syndrome, 4 with patent foramen ovale (PFO) being the most common cause of intracardiac shunting, followed by atrial septal defects and atrial septal aneurysms.…”
Section: Introductionmentioning
confidence: 99%
“…The most frequent cause is interatrial shunting via an AIC or PFO. When right atrial pressure is higher than left atrial pressure under conditions such as pericardial disease, PE or right heart infarction, a right-to-left shunt across the defect occurs [10] [11]. However, when there are anatomical and functional components that direct venous blood flow to the AIC or PFO, as in patients with a prominent Eustachian valve or aortic elongation or dilatation, a right-to-left shunt can occur even in the absence of elevated right atrial pressure [12].…”
Section: Discussionmentioning
confidence: 99%
“… 4 The most common cause is interatrial shunting through an ASD or PFO. 5 , 6 When the right atrial pressure is higher than the left atrial pressure in conditions such as pericardial disease, pulmonary embolism, or right heart infarction, right-to-left shunting through the defect occurs. However, when there are anatomical and functional components that direct venous blood flow toward the ASD/PFO, as in patients with a prominent Eustachian valve 7 or aortic elongation or dilatation, 8 right-to-left shunting can occur even in the absence of elevated right atrial pressure.…”
Section: Discussionmentioning
confidence: 99%