“…POS remains a rare clinical condition defined by dyspnea and desaturation in the upright position with resolution of these signs and symptoms in the supine position. 1,2 The desaturation, orthodeoxia, has several definitions including a drop in partial pressure of oxygen by more than 4 mmHg or a decrease in saturation by more than 5% from the upright to supine positions 3 or saturation less than 90% or partial oxygen pressure less than 60 mmHg in upright position with normalization in lying position. 10 About 87% of patients with POS have intracardiac shunting, most commonly from patent foramen ovale.…”
Section: Discussionmentioning
confidence: 99%
“…3 Our patient had POS in the context of physiological right-to-left shunt without pulmonary arterial hypertension. 8,9 PLSVC is a rare congenital anomaly, most frequently diagnosed incidentally with an incidence of 0.3% in general population. 11 The incidence is higher in patients with congenital heart disease reaching 4%.…”
Section: Discussionmentioning
confidence: 99%
“…Platypnea-Orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by shortness of breath and hypoxia in the upright position that is relieved with supine positioning. 1,2 The etiologies of POS include intracardiac right-to-left shunt, extracardiac abnormalities like pulmonary arterio-venous malformation, hepato-pulmonary syndrome and ventilation/perfusion mismatch. 3,4,5,6 On very rare occasion, a persistent left superior vena cava (PLSVC) may lead to POS by draining into left atrium and causing physiological right-to-left shunt leading to hypoxia and dyspnea.…”
Platypnea-Orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by shortness of breath in the upright position that is relieved with supine positioning. We present a rare case of persistent left superior vena cava (PLSVC) draining into the left atrium causing a right-to-left shunt and subsequent POS. A 30-year-old female with a past medical history of hypertrophic cardiomyopathy, congenital Long QT syndrome and a left-sided dual chamber implantable cardioverter-defibrillator (ICD) presented with dyspnea and lightheadedness. Prior to presentation, the patient underwent a leftsided ICD extraction due to ICD lead infection and re-implantation from the right side through the cephalic vein. After further investigation, it was concluded that the PLSVC resulted in a physiological right-to-left shunting causing POS, with resolution of her symptoms after surgical ligation. To our knowledge, this is the first case report of PLSVC presenting with POS without anatomical intracardiac shunts following iatrogenic right superior vena cava (RSVC) obstruction.
“…POS remains a rare clinical condition defined by dyspnea and desaturation in the upright position with resolution of these signs and symptoms in the supine position. 1,2 The desaturation, orthodeoxia, has several definitions including a drop in partial pressure of oxygen by more than 4 mmHg or a decrease in saturation by more than 5% from the upright to supine positions 3 or saturation less than 90% or partial oxygen pressure less than 60 mmHg in upright position with normalization in lying position. 10 About 87% of patients with POS have intracardiac shunting, most commonly from patent foramen ovale.…”
Section: Discussionmentioning
confidence: 99%
“…3 Our patient had POS in the context of physiological right-to-left shunt without pulmonary arterial hypertension. 8,9 PLSVC is a rare congenital anomaly, most frequently diagnosed incidentally with an incidence of 0.3% in general population. 11 The incidence is higher in patients with congenital heart disease reaching 4%.…”
Section: Discussionmentioning
confidence: 99%
“…Platypnea-Orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by shortness of breath and hypoxia in the upright position that is relieved with supine positioning. 1,2 The etiologies of POS include intracardiac right-to-left shunt, extracardiac abnormalities like pulmonary arterio-venous malformation, hepato-pulmonary syndrome and ventilation/perfusion mismatch. 3,4,5,6 On very rare occasion, a persistent left superior vena cava (PLSVC) may lead to POS by draining into left atrium and causing physiological right-to-left shunt leading to hypoxia and dyspnea.…”
Platypnea-Orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by shortness of breath in the upright position that is relieved with supine positioning. We present a rare case of persistent left superior vena cava (PLSVC) draining into the left atrium causing a right-to-left shunt and subsequent POS. A 30-year-old female with a past medical history of hypertrophic cardiomyopathy, congenital Long QT syndrome and a left-sided dual chamber implantable cardioverter-defibrillator (ICD) presented with dyspnea and lightheadedness. Prior to presentation, the patient underwent a leftsided ICD extraction due to ICD lead infection and re-implantation from the right side through the cephalic vein. After further investigation, it was concluded that the PLSVC resulted in a physiological right-to-left shunting causing POS, with resolution of her symptoms after surgical ligation. To our knowledge, this is the first case report of PLSVC presenting with POS without anatomical intracardiac shunts following iatrogenic right superior vena cava (RSVC) obstruction.
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