Platypnea-orthodeoxia Syndrome Research Articles

767 NEVER TOO LATE TO SHOW-UP: A RARE CASE OF PLATYPNEA-ORTHODEOXIA SYNDROME

Abstract Patient presentation. We present the case of an 85-years old woman, hypercholesterolemic, with a previous history of microvascular angina treated with nitrates, ASA and ranolazine. She presented in our emergency department for repeated episodes of seizure and “drop attacks” and she underwent neurological and cardiological evaluation. ECG showed sinus tachycardia, 110/min, left anterior hemiblock. Transthoracic echocardiogram was normal. Head CT-scan and cerebral magnetic resonance documented mild hyperintense areas in the subcortical part of white matter suggestive for chronic cerebrovascular disease. Carotid Doppler was negative for significant stenosis. Thus, suspecting an unexplained syncope, she underwent loop recorder implantation. One month later she was admitted to the emergency room for dysarthria, left hemiparesis and ataxia, that completely regressed after 24-hours, posing the diagnosis of transient ischemic stroke. Physical examination was normal. During in-hospital stay, the patient progressively developed dyspnoea and oxygen desaturation (SpO2 < 90%). Initial work-up. Due to oxygen desaturation non-invasive ventilation with Venturi mask (60%) was started. Blood tests were normal. The arterial blood gas analysis in Venturi mask showed: pH 7.47, pCO2 48 mmHg, pO2 136 mmHg, P/F 226. Despite non-invasive ventilation with Venturi mask, P/F remained low and so continuous positive airway pressure (CPAP) was started. A repeated echocardiogram showed a mild reduction of ejection fraction (45-50%) due to hypokinesia of the septum, secondary to right ventricular overload and compression. Pulmonary embolism was excluded by angio-CT. Medical management with vasodilators and diuretics finally allowed to wean off the non-invasive ventilation, still maintaining oxygen support with nasal cannulas. However, surprisingly, the mobilization of the patient in a sitting position resulted impossible, due to the occurrence of sudden desaturation. Diagnosis and Management. Suspecting a manifestation of platypnea-orthodeoxia syndrome (POS) a transoesophageal echocardiogram was performed. It revealed a wide patent foramen ovale (PFO), maximum diameter 1.5 cm, with a baseline mild right-to-left shunt, confirmed with sonicated contrast, that increased in the sitting position. Therefore, the diagnosis of POS syndrome and paradoxical cerebral embolization was posed. Elective PFO closure with the percutaneous positioning of an atrial septal defect (ASD) Occluder n.15 was successfully performed. Conclusions The platypnea-orthodeoxia syndrome is a rare cause of hypoxemia. The most common structural abnormality associated with this syndrome is the PFO, which can remain asymptomatic for decades. A shift in the relationship between right-and left intra-cardiac pressures, a change in the anatomy of the adjoining structures with age, stretching the atrial septum and favouring the blood inflow into the PFO (as ascending aorta dilatation or Thoracic Kyphosis) can lead to POS. The closure of the atrial defect is generally resolutive. However, the heterogeneous clinical presentations, the rarity of the disease and the confounding role of comorbidities can often result misleading, especially in advanced age.

Acute Onset of Refractory Hypoxemia: A Rare Hemodynamic Cause of Dyspnea

Platypnea-orthodeoxia syndrome (POS) is a rare condition of positional dyspnea with hypoxemia that can pose a diagnostic challenge to clinicians. We report two cases of POS with different pathophysiologic triggers and similar clinical features. In our report, both patients manifested a severe hypoxemia with low response to increase of fractional inspired oxygen, unremarkable physical examination of the lungs, and no radiological findings of the pulmonary embolism. In both cases, an interatrial communication was found in association with a right-to-left interatrial shunting. Recognition of POS is crucial because symptoms can be quickly relieved by recumbency, unlike other types of hypoxemia. This simple solution may prevent the use of sedative therapy, invasive ventilatory support, and vasopressor agents, which could even exacerbate the right-to-left extrapulmonary shunt and worsen clinical conditions.

Patent Foramen Ovale-Induced Platypnea-Orthodeoxia Syndrome: A Case Report and Literature Review

Platypnea-orthodeoxia syndrome (POS) is a rare condition characterized by dyspnea and hypoxia worsening in the upright position and relieved in the recumbent position. POS can result from anatomical or functional conditions that cause interatrial communication or deformity of the atrial septum. Investigations with imaging and cardiac catheterization can aid in the evaluation. In cases where POS is caused by intracardiac shunting without pulmonary hypertension, closure of the intracardiac shunt can be curative. We report a case of POS in a 54-year-old male who was treated successfully with percutaneous closure of a patent foramen ovale (PFO).

Mechanisms of platypnea-orthodeoxia syndrome

Mechanisms of platypnea-orthodeoxia syndrome

ARDS with Concomitant Pneumomediastinum-Pneumothorax Presenting as Platypnoea-Orthodeoxia Syndrome

ARDS with Concomitant Pneumomediastinum-Pneumothorax Presenting as Platypnoea-Orthodeoxia Syndrome

Platypnoea Orthodeoxia Syndrome and Patent Foramen Ovale Closure: Single-Centre Experience and Long-Term Follow-Up.

Platypnoea orthodeoxia syndrome (POS) is an uncommon condition characterised by dyspnoea and arterial desaturation induced by an upright position and relieved in the supine position, usually due to a patent foramen ovale (PFO). Percutaneous closure of a PFO is the preferred treatment to cure POS. This study aimed to evaluate the clinical and gasometrical characteristics and to describe the long-term outcomes of percutaneous PFO closure in a group of patients with POS. Patients with POS and a PFO treated by percutaneous intervention from 2010-2020 were reviewed. The primary efficacy outcome was the arterial oxygen pressure to fraction of inspired oxygen (PaO2/FiO2) ratio before and 24 hours after the procedure. Total clinical success was considered if the arterial oxygen saturation measured by pulse oximetry (SpO2) improved to >94% in the supine and sitting positions without supplemental oxygen, while partial success was considered if SpO2 improved from baseline but still required oxygen to achieve >94%. Secondary outcomes were an absolute improvement in SpO2 and sense of dyspnoea, without significant residual shunt on transthoracic echocardiography (TTE) at follow-up. Of 168 patients undergoing PFO or atrial septal defect closure, 14 had POS (8.3%). Percutaneous PFO closure was successfully performed in all patients with a single device. Twelve of 14 patients had total clinical success (86%) and one patient had partial success. The PaO2/FiO2 ratio increased from 155.9±50.6 to 318.3±73.4 after PFO closure (p=0.002). All patients with total clinical success had a successful secondary efficacy outcome with an absolute improvement in SpO2 and complete resolution of dyspnoea, which was maintained at follow-up (37±20 months; range, 11 months to 6 years). None had a significant residual shunt between 12 and 24 months of follow-up. The PFO percutaneous closure was a successful, durable and safe method for patients presenting with POS; it achieved major improvements in both gasometrical parameters and quality of life.

Two Cases of Platypnea-orthodeoxia Syndrome (POS) in Elderly Patients

Two Cases of Platypnea-orthodeoxia Syndrome (POS) in Elderly Patients

Severe Pneumonia Caused by SARS-CoV-2: A Novel Cause of Platypnoea-Orthodeoxia Syndrome.

Platypnoea-orthodeoxia syndrome (POS) is a condition characterized by dyspnoea and hypoxaemia while sitting or standing, which improves during decubitus. It is usually caused by intracardiac right-to-left shunting through a patent foramen ovale but may also occur due to pulmonary ventilation-perfusion mismatch of other aetiologies. A new cause of POS was recently described: SARS-CoV-2 pneumonia. We report the case of a 62-year-old man admitted for SARS-CoV-2 pneumonia with respiratory failure. Chest computed tomography angiography showed pulmonary thromboembolism and parenchymal lung changes compatible with COVID-19. He had worsening dyspnoea in a sitting position, relieved by assuming the dorsal position. He was diagnosed with POS after other causes were excluded. POS is an underdiagnosed complication of COVID-19 and is manageable with respiratory rehabilitation.LEARNING POINTSPlatypnoea-orthodeoxia syndrome is an under-recognized condition presenting as a complication of a structural shunt.We describe SARS-CoV-2 pneumonia as a novel cause for this syndrome.It is a reversible syndrome provided there is early diagnosis and initiation of pulmonary rehabilitation.

Platypnea-orthodeoxia syndrome in a patient with ongoing COVID-19.

Key messageSARS‐CoV‐2 infection of the vascular endothelium causes excessive vasodilation. It is important in the rehabilitation of patients with COVID‐19 to recognize that increased blood flow in lung lesions at the base of the lung due to vasodilation may cause V/Q mismatch and result in platypnea‐orthodeoxia syndrome.

0829 Workup of Nocturnal Hypoxemia Leads to Diagnosis of Patent Foramen Ovale

Abstract Introduction Patent foramen ovale (PFO) in adults often remains asymptomatic until clinical manifestations such as cryptogenic stroke, migraine headache, air embolism, hypoxemia, or platypnea-orthodeoxia syndrome occur. In this case, prior workup of cryptogenic stroke failed to identify a PFO that was diagnosed after further investigation of polycythemia which revealed nocturnal hypoxemia on polysomnogram. Report of Cases: A 60-year-old male with history of recurrent venous thromboembolic events (VTE), secondary polycythemia, and cryptogenic strokes was referred for a polysomnogram during the evaluation of polycythemia. Over the span of six years, he had multiple cryptogenic strokes and VTEs in the setting of polycythemia and normal hypercoagulability labs. Further evaluation suggested secondary polycythemia after serum erythropoietin and JAK2 mutation testing were negative. The patient was a never smoker without evidence of malignancy or renal disease leading to suspicion that his polycythemia was due to hypoxemia despite normal oxygen saturations during point of care evaluations. He frequently reported shortness of breath during appointments. During a polysomnogram, the patient was found to have a total apnea-hypopnea index of fewer than 5 events per hour but demonstrated hypoxemia with 49.7% of total sleep time spent below an oxygen saturation of 90%. He was referred to pulmonology for further evaluation that showed normal resting oxygen saturation, no desaturation on a six-minute walk test, mild restrictive defect on pulmonary function testing, and a normal chest computed tomography with angiography. Prior transthoracic echocardiogram had demonstrated an atrial septal aneurysm without communication between the atrial chambers. No evidence suggestive of a right-to-left shunt was found on lateral imaging of the brain during a ventilation-perfusion scan. Further evaluation of the aneurysmal atrial septum on transesophageal echocardiogram with an agitated saline bubby study demonstrated a patent foramen ovale with a right-to-left shunt. In the setting of hypoxia leading to secondary polycythemia, the patient was scheduled for PFO closure. Conclusion Hypoxemia out of proportion to sleep-disordered breathing on polysomnogram should prompt further evaluation. Despite multiple prior strokes, our patient’s PFO had gone undiagnosed until polycythemia prompted a polysomnogram that demonstrated isolated nocturnal hypoxemia and prompted a further workup. Clinically significant hypoxemia is an indication for PFO closure. Support (If Any)

Platypnea-orthodeoxia syndrome in COVID-19: rare, not uncommon

COVID-19 is a contagious disease caused by the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). As humanity continues to fight this virus, it keeps presenting with new manifestations and complications. Platypnea-orthodeoxia syndrome (POS) is a rare clinical syndrome characterized by dyspnea and oxygen desaturation, in the supine to sitting position, which resolves with recumbency and can be diagnosed with a simple pulse oximeter. We report three cases of COVID-19 pneumonia, who developed POS during the recovery phase, in the absence of pulmonary hypertension.

Platypnoea-orthodeoxia syndrome as an uncommon cause of dyspnoea: a literature review.

Platypnoea–orthodeoxia syndrome (POS) is an uncommon but challenging clinical condition characterised by positional dyspnoea (platypnoea) and arterial desaturation (orthodeoxia) in the upright position that improve in the supine position. Since its first description, many cases have been reported and many conditions have been associated with this syndrome. Herein, we review the clinical presentation, pathophysiology, diagnostic work‐up and management of patients with POS, aiming to increase the awareness of this often misdiagnosed condition.

Platypnea-orthodeoxia Syndrome: An Important Cause of Morbidity in Post Coronavirus Disease Patients.

Platypnea-orthdeoxia syndrome (POS) is a clinical scenario where patient get breathless while sitting or standing. Its important causes are cardiac shunts, hepatopulmonary syndrome and pulmonary ventilation perfusion mismatch. During this pandemic as cases of pulmonary fibrosis increased, we find POS as one of the important cause of morbidity during recovery. Early recognition of this will decrease the morbidity and unrealistic expectation of fast recovery.How to cite this articleBhushan D, Kumar V, Sahoo BH, Hegde A. Platypnea-orthodeoxia Syndrome: An Important Cause of Morbidity in Post Coronavirus Disease Patients. Indian J Crit Care Med 2022;26(3):401–402.

Clinical and echocardiographic features of platypnea-orthodeoxia syndrome: a single-centre experience

Abstract Funding Acknowledgements Type of funding sources: None. Background Platypnea-orthodeoxia syndrome (POS) is an uncommon condition characterized by dyspnoea and hypoxemia in the upright position that improves with recumbency.1 Possible causes are intracardiac shunt, pulmonary arteriovenous shunt, and V/Q mismatch.1,2 Echocardiography is the cornerstone of POS diagnosis, with special focus on atrial septal defect (ASD) morphology and right-to-left shunt confirmation.3 Purpose To evaluate the clinical and echocardiographic features of patients presenting with POS due to a cardiac cause. Methods We performed a retrospective analysis of patients diagnosed with POS in our centre between 2015 January and 2021 August. Routine blood tests and transthoracic echocardiogram (TTE) were performed in all patients. Demographics, clinical presentation, blood test results, TTE information, and PFO closure procedure details were recorded. Results Seven patients were included, 85.7% female (n = 6). The median (IQR) age was 78 (72-85) years. The most prevalent cardiovascular risk factors were hypertension (100%; n = 7) and overweight/obesity (85.7%; n = 6). Two patients (28.6%) had chronic pulmonary disease. The most common symptoms were fatigue and exercise intolerance (n= 5; 71.4%) and the most frequent sign was persistent hypoxemia (n = 7; 100%), although 28.6% (n = 2) patients did not present the typical positional changes in peripheral oxygen saturation. Haemoglobin levels [14.1 (13.3-15.2)] were within the normal range and serum NTproBNP levels [656 (287-1196)] were slightly elevated. Left ventricle function was preserved in all patients; right ventricle morphology and function were normal in 85.7% (n = 6) patients, low probability of pulmonary hypertension in TTE was found in 85.7% (n = 6), and exuberant Eustachian valve was observed in 14.3% (n = 1). All patients presented atrial septal hypermobility, 87.5% (n = 6) meeting atrial septal aneurysm criteria. Patent foramen ovale was found in 85.7% of patients (n = 6) and ostium secundum ASD in 14.3% (n = 1). POS precipitating factors were aortic root dilation (28.6%; n = 2), chest trauma (14.3%; n = 1), right hip arthroplasty (14.3%; n = 1), atrial septal stretching regarding right volume overload (14.3%; n = 1). The underlying mechanism was unknown in 28.6% (n = 2) of patients. ASD closure was performed in 57.1% (n = 4) of patients: 75% (n = 3) showed residual shunt, but clinical improvement was reported by all. No acute complications were described, except for paroxysmal atrial fibrillation (14.3%; n = 1). Conclusion POS diagnosis depends on high clinical suspicion: the most common manifestations are fatigue and persistent hypoxemia. Typical positional changes in oxygen saturation are not present in all patients. Polycythaemia, right chambers dilation, and pulmonary hypertension are not common. Echocardiography is fundamental for diagnosis, allowing right-to-left shunt confirmation and ASD morphology evaluation to outline a successful closure procedure.

A case of interstitial pneumonia with platypnea-orthodeoxia syndrome and difficult in early mobilization

A case of interstitial pneumonia with platypnea-orthodeoxia syndrome and difficult in early mobilization

Patent foramen ovale in children: Unique pediatric challenges and lessons learned from adult literature.

ABSTRACTA patent foramen ovale (PFO) is a frequent incidental finding during echocardiography in otherwise healthy children. In most healthy children with a diagnosis of isolated incidental PFO, no further follow-up or intervention is necessary. In some children, PFO is associated with certain clinical syndromes such as cryptogenic stroke, decompression sickness, migraine, and platypnea–orthodeoxia syndrome. This review discusses PFO anatomy, diagnostic imaging, PFO-associated clinical situations, management options, and the role of PFO in certain congenital heart disease. This review also highlights the current deficiency of pediatric data guiding management of these uncommon but important PFO-associated conditions. Future multicenter randomized controlled studies are necessary to guide the management of these unique and challenging PFO-associated conditions.

A Closed Case of Platypnoea-Orthodeoxia Syndrome

Patent foramen ovale (PFO) do not typically cause systemic hypoxaemia in the absence of pulmonary or right heart pathology causing raised right atrial pressures.

Stroke and Respiratory Failure: Mind the Shunt!

When stroke patients present with respiratory failure, the first thought that clinicians have is that it is probably related to aspiration pneumonia. However, other causes should be considered, such as intracardiac or intrapulmonary shunts, that could present with paradoxical embolism. Paradoxical embolism is a rare entity defined by the occurrence of a venous thrombotic event associated with a systemic arterial embolism. Frequently, paradoxical embolism presents with platypnoea-orthodeoxia syndrome. Platypnoea-orthodeoxia syndrome is uncommon and is characterized by dyspnoea and hypoxaemia induced by orthostatic position, where symptoms and oxygenation are relieved by recumbency. The authors report a case of a patient who presented with an ischaemic stroke and progression to platypnoea-orthodeoxia syndrome with documentation of simultaneous pulmonary embolism and pulmonary arteriovenous malformations.LEARNING POINTSThere are other causes to explain simultaneous presentation of stroke and respiratory failure, which should be kept in mind.The association between arterial and venous thrombosis; be aware of paradoxical embolism and search for intracardiac or intrapulmonary shunts.Platypnoea-orthodeoxia syndrome is characterized by dyspnoea and hypoxaemia induced by orthostatic position and relieved by recumbency; it is a red flag to think of an intracardiac or intrapulmonary shunt.

486 A blue man standing

An 85-year-old was admitted in Internal Medic Ward for recurrent episodes of acute respiratory failure. His electrocardiogram showed a new onset of atrial flutter with spontaneous reversal to sinus rhythm (narrow QRS complex and normal atrioventricular conduction). The dyspnoea was worse with the patient sitting and was better when lying supine. Also, he report back pain associated to his major kyphoscoliosis. Results of chest radiography and blood test were normal. The arterial blood gas test revealed a hypocapnic hypoxemic respiratory failure, therefore the patient oxygen supplementation with noninvasive ventilation (NIV) was started, but peripheral oxygen saturation was persistently below 88% despite the high flow oxygenation. The patient underwent nasopharyngeal (NP) swab (ruling out SARS-CoV-2), high-resolution computed tomography (which was not pathologic), and computed tomography angiography (excluding pulmonary embolism). A transthoracic echocardiography was performed showing concentric hypertrophy, left atriomegaly and severe aortic ectasia of the root and of his ascending part, normal pulmonary pression and an aneurism of the interatrial septum with the presence of right-to-left shunt after using agitate saline contrast with Valsalva maneuver. Transesophageal echocardiography (TTE) confirmed the presence of severe structural interatrial septal abnormality with wide left convex aneurysm and an atrial septum defect (ASD) as ostium secundum, causing severe bidirectional shunt. Therefore, the patient underwent a successful transcatheter closure of secundum ASD with device Amplatzer Septal Occluder 018, while monitored with cardiac catheterization and transesophageal echocardiography. After few days, the patient was discharged at home without oxygen therapy. At the 3-month follow-up visit he reported no respiratory symptoms. Platypnea-orthodeoxia syndrome (POS) is an uncommon disorder characterized by dyspnoea and hypoxemia that occurs when the patient is sitting or standing and disappear quickly when recumbent. POS is characterized by both an anatomic and a functional component. The anatomic element is typically an interatrial communication such as ASD or Patent Foramen Ovale, which cause blood shunt left-to-right due to a higher pressure in left atrium and a greater compliance of the right ventricle, in the presence of normal pulmonary artery pressure. The syndrome occurs when a functional element, such as an increased atrial pressure or a decreased compliance of the right ventricle, reverses the flow. The shunts are often small and could remain asymptomatic: the high left atrial pressure let the defect close until there is a reverse pression which stretched atrial septum in particular in the upright position. In fact, when the patient is standing, the inferior vena cava comes in line with the defect increasing the right-to-left flow, sparking the respiratory symptoms. This could be linked to a cardiac or an extracardiac condition; in this case the presence of kyphoscoliosis and severe aortic ectasia of the root and of his ascending part, played an important role. The diagnosis is mainly made by echocardiography and cardiac catheterization to verify the mismatch in oxygen saturation between the pulmonary vein and the aorta. The diagnosis of POS is challenging, often considered only after other possible diagnosis is excluded. When POS is triggered by an interatrial defect in the absence of severe pulmonary hypertension, the usual treatment is a percutaneous or surgical closure.

79 Platypnea–orthodeoxia syndrome as an uncommon cause of dyspnoea in a nonagenarian with aortic dissection

Abstract Methods and results Platypnea–Orthodeoxia syndrome (POS) is an uncommon but challenging clinical condition characterized by positional dyspnoea (platypnea) and arterial desaturation (orthodeoxia) in the upright position that improve in the supine position. POS can occur insidiously, as progressive unexplained oxygen desaturation for months or years, or as acute life-threatening unexplained hypoxaemia. The most common cause is the presence of an intracardiac shunt (patent foramen ovale and other interatrial defects) associated with a secondary anatomic or functional defect that serve as substrate for a right to left intracardiac shunt leading to oxygen desaturation. Conclusions Herein we present the case of a nonagenarian with a known history of ascending aortic aneurysm, that was admitted to the emergency department of our institution with a complaint of intermittent chest pain and dyspnoea. POS was then diagnosed, and the patient underwent a successful percutaneous closure with an Amplatzer™ device (25/25 mm) with minimal residual right-to-left shunt leading to a significant relief of the dyspnoea and improvement of arterial saturation. We also herein revise the clinical presentation, pathophysiology, diagnostic work-up, and management of patients with POS, aiming at increasing the awareness of this uncommon but often misdiagnosed treatable condition.