Project description: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.
Project description:Platypnea-orthodeoxia syndrome is a rare entity seen in patients with patent foramen ovale, characterized by dyspnea and arterial desaturation in the upright position. We describe a case of a patient who presented with cryptogenic stroke, evidence of right-to-left interatrial shunting, and refractory hypoxemia necessitating closure of his patent foramen ovale.
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.
Project description:An 82-year-old man developed acute breathlessness and cyanosis, exacerbated while upright and improved on lying flat (platypnea-orthodeoxia syndrome). Echocardiography revealed acute torrential tricuspid regurgitation due to a flail posterior leaflet leading to right-to-left shunting through a patent foramen ovale (PFO). The patient's symptoms resolved after transcatheter PFO closure and tricuspid valve edge-to-edge repair.
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.
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.
Project description:Platypnea-orthodeoxia syndrome (POS) attributed to patent foramen ovale (PFO) can be caused by a variety of clinical conditions. A 70-year-old woman was admitted to our hospital for further evaluation of POS. Her symptoms developed along with the spread of infiltrative shadows in both lower lung fields during the preceding 2 years. Contrast transthoracic echocardiography with agitated saline revealed grade III intracardiac right-to-left shunting, presumably across a PFO. Transesophageal echocardiography demonstrated severe tricuspid regurgitation (TR) caused by the prolapse of the anterior leaflet. Bidirectional shunt flow, mainly from right-to-left across a PFO, that increased in the sitting position was also observed. She was diagnosed as having PFO associated with severe primary TR. Therefore, tricuspid valve repair and direct PFO closure were performed. Her symptoms resolved completely soon after the operation and her oxygen saturation was maintained. This patient's disease seemed to have worsened with the spread of pulmonary parenchymal involvement, which caused ventilation-perfusion mismatch and elevation of alveolar pressures. Echocardiography is an essential imaging modality in addition to other diagnostic examinations and imaging studies when assessing the pathogenesis in patients with POS.Learning objectivePlatypnea-orthodeoxia syndrome (POS) associated with patent foramen ovale may be caused by a variety of clinical conditions, and POS in our patient may be caused by the worsening of pulmonary parenchymal involvement. Examinations to evaluate all causes of POS are essential for making the diagnosis. Contrast transthoracic echocardiography was useful in assessing the cause of POS.
Project description:BackgroundPlatypnea-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 summaryA 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.DiscussionThis 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.