A Rare Case of Platypnea-Orthodeoxia Syndrome in a Patient With Undiagnosed Atrial Septal Defect.

Platypnea-orthodeoxia syndrome (POS) is a rare condition characterized by dyspnea and oxygen desaturation that worsens in the upright position and improves when lying down. We report the case of a 67-year-old male who presented with a 14-month history of dyspnea in the sitting/standing position. Despite treatment for suspected asthma, his symptoms persisted, and he was referred to our hospital for further evaluation. Physical examination and arterial blood gas analysis confirmed the presence of POS, with a significant decrease in PaO2 and SpO2 when moving from a supine to an upright position. Contrast-enhanced CT showed no obvious embolism nor arteriovenous fistula, and ventilation-perfusion scintigraphy demonstrated ventilation-perfusion mismatch with a right-to-left shunt fraction of 9.4%, without any focal defect. Transthoracic echocardiography with a microbubble test demonstrated a right-to-left shunt that increased in the upright position. Transesophageal echocardiography revealed an atrial septal defect (ASD) with an atrial septal aneurysm and the presence of an inferior vena cava valve, causing a bidirectional shunt. The patient was diagnosed with POS secondary to ASD and was referred for percutaneous closure of the defect. Following the procedure, the shunt resolved, and the patient's orthostatic oxygen desaturation improved. This case highlights the importance of considering POS in patients with positional dyspnea and the value of performing diagnostic tests, such as echocardiography, in different positions to identify the underlying cause. Early recognition and appropriate management of POS can significantly improve patients' quality of life and prevent complications associated with chronic hypoxemia.

Updates on Patent Foramen Ovale (PFO) Closure.

PURPOSE OF REVIEW: Patent foramen ovale (PFO) has been previously linked to left circulation thromboembolism and stroke. This review article aims to discuss the latest evidence, updated societal guidelines, diagnostic algorithms and novel therapeutic devices for PFO closure. RECENT FINDINGS: PFO closure for cryptogenic stroke and systemic embolization is supported by a large body of evidence and has a strong societal recommendation. Limited data are available for platypnea-orthodeoxia syndrome, although closure appears to be beneficial. Current data do not support routine closure for migraines and decompression Illness. Development of heart-brain teams can improve identification of patients most likely to benefit from closure, utilizing a combination of imaging test and risk score algorithms. Multiple novel devices aiming at reducing complications and improving the long-term impact of current available devices are being evaluated. PFO closure has significantly progressed over the last years, with new data supporting its superiority in reducing risk of recurrent embolic stroke in patients with PFO-related stroke. Additional clinical data are required to provide further refinements on patient selection and guidance on treatment of specific subgroups.

Atrial septal defect and platypnea-orthodeoxia syndrome: usefulness of the balloon occlusion test.

A 37-year-old man was referred to our medical center with a diagnosis of Eisenmenger syndrome due to an atrial septal defect (ASD). At admission he had central cyanosis, acrocyanosis, and progressive effort dyspnea; his symptoms improved during lying position.

Prevalence of Aortic Root Pathologies in Platypnea-Orthodeoxia Syndrome Secondary to Intra-Cardiac Shunts.

Background: Atrial septal defects can allow right to left shunting of venous blood which presents clinically as platypnea-orthodeoxia syndrome. It is believed that concomitant presence of aortic root pathologies increases the likelihood of shunting. Methods: The study included a review of 510 articles listed in PubMed of patients with platypnea-orthodeoxia syndrome. Case reports of patients with extra-cardiac etiologies of platypnea-orthodeoxia were excluded. Results: We reviewed 191 case reports, and 98 cases (51.3%) had evidence of concomitant aortic root pathology. Furthermore, of the remaining 93 case reports, 69 ones excluded any mention of the nature of the aortic root altogether, further suggesting that this is an underreported number. Conclusions: There is a high prevalence of aortic root pathologies in patients with platypnea-orthodeoxia syndrome secondary to intra-cardiac shunts. In patients with unexplained hypoxemia and incidental finding of aortic root pathology, it may be worthwhile to obtain postural oxygen saturation measurements to exclude intra-cardiac shunts as the potential cause.

Patent Foramen Ovale: The Unresolved Questions.

Patent Foramen Ovale (PFO) is a remnant of fetal circulation that could be observed in the 25% of the population worldwide. PFO is associated to numerous clinical conditions as migraines, coronary embolization, transient ischemic attacks, and stroke. The main PFO concerns are related to its correlation to stroke, in particular in young adults. Despite the impact on morbidity that PFO could have, to date there is not clear evidence about its management and treatment. In this narrative review our aim is to summarize the more recent evidence in the literature dealing with PFO, in order to provide an updated overview on this topic.

Platypnea-Orthodeoxia Syndrome and COVID-19 Successfully Treated With Percutaneous Patent Foramen Ovale Closure: A Report of Two Cases and Literature Review.

Platypnea-orthodeoxia syndrome (POS) is a rare clinical condition characterized by positional dyspnea and/or hypoxia. We report two cases of patients with COVID-19 bronchopneumonia with a torpid evolution. Due to clinical suspicion of POS, a diagnostic workup was performed, including a bubble echocardiography, which revealed a patent foramen ovale (PFO) with early and massive passage of bubbles to the left cavities. Both patients underwent percutaneous PFO closure with a resolution of POS. Here, we present the second and third cases of POS associated with PFO successfully closed during the acute phase of COVID-19. This suggests that PFO closure could be a potential treatment option for this condition.

Platypnoea-orthodeoxia syndrome in COVID-19 pneumonia patients: An observational study.

This retrospective observational study aimed to assess the clinical characteristics of platypnea-orthodeoxia syndrome in patients with coronavirus disease 2019 (COVID-19) treated using mechanical ventilation or high-flow nasal canula. We analyzed 42 consecutive patients with COVID-19 from January 2020 to March 2022. The primary outcomes were the incidence of platypnea-orthodeoxia syndrome, the time with required long-term oxygen therapy, and short-term prognosis. Additionally, we examined the relationships between platypnea-orthodeoxia syndrome and COVID-19 severity, the time with long-term oxygen therapy, and short-term prognosis. Of the 42 included patients, 15 (35.7 %) had platypnea-orthodeoxia syndrome. Although mortality was not significantly different between both groups, the oxygen withdrawal rate in the platypnea-orthodeoxia syndrome group was significantly lower than that in the group without this syndrome. Clinical staff should be aware of the possibility of platypnea-orthodeoxia syndrome during positional changes in patients with COVID-19. Recognizing POS can improve early detection, countermeasures, and safety during physiotherapy.

Coronary air embolism during transcatheter patent foramen ovale closure for platypnea-orthodeoxia syndrome in a patient with severe respiratory disorder: a case report.

Background: Coronary air embolism (CAE) is a rare and life-threatening complication of endovascular procedures, mostly due to procedure-related causes. Case summary: A 70-year-old man with severe respiratory disorder presented with patent foramen ovale (PFO)-related platypnea-orthodeoxia syndrome (POS). Transcatheter PFO closure was performed under local anaesthesia and intracardiac echocardiographic guidance. After a 5-Fr catheter was passed through the PFO via a 7-Fr femoral vein sheath, the patient suddenly coughed and breathed deeply. Thereafter, intracardiac echocardiography showed massive microbubbles in all cardiac chambers and the ascending aorta, and an electrocardiogram showed ST-segment elevations in the anterior and inferior leads. Emergency coronary angiography confirmed occlusion of the mid-left anterior descending artery, suggesting CAE. As the intracoronary infusion of saline, nitroglycerine, and nicorandil was ineffective, we performed air aspiration using a thrombectomy device, achieving coronary blood flow improvement and ST-segment resolution. Thereafter, positive pressure support using manual bag-valve-mask ventilation under intravenous sedation supported successful transcatheter PFO closure without further air embolization. Discussion: In this case with severe respiratory dysfunction, spontaneous deep breathing (spontaneous Valsalva manoeuvre) caused negative intrathoracic pressure and large drops in intravascular pressure. This phenomenon might have induced air contamination during device advancement, either by entrapping or leaving residual air in the gaps between the catheter and the sheath. Additionally, PFO with right-to-left shunts is more likely to cause paradoxical air embolization. Thus, the spontaneous Valsalva manoeuvre should be avoided with appropriate respiratory management to prevent paradoxical air embolization, including CAE, during transcatheter PFO closure under local anaesthesia in severe respiratory dysfunction patients.

Association between Covid-19 infection and platypnea-orthodeoxia syndrome.

Introduction and importance: Platypnea-orthodeoxia syndrome is defined as dyspnoea and deoxygenation when changing from a recumbent to an upright position. Post-Covid-19 sequelae can induce or exacerbate pulmonary hypertension and thereby render a previously mild and asymptomatic platypnea-orthodeoxia syndrome to manifest with new or worsening symptoms. Case presentation: The authors present the case of an 80-year-old man who following an episode of moderate-severe Covid-19 infection developed type I respiratory failure that required hospital discharge with long-term oxygen therapy. He had a background history of postural paroxysmal hypoxaemia which had previously raised the suspicion of a right-to-left shunt through either a patent foramen ovale, atrial septal defect or an intrapulmonary arteriovenous malformation. However, given the low burden of symptoms this was not explored further. Following recovery from Covid-19 infection, the patient experienced marked dyspnoea and oxygen desaturation in an upright position that was relieved by a return to a supine position. Discussion and conclusion: Persistent dyspnoea and hypoxia are common symptoms in patients who experience post-Covid-19 syndrome. However, when patients with prior moderate-to-severe Covid-19 illness present with new onset breathlessness and/or desaturation that is worsened in an upright position, platypnea-orthodeoxia syndrome should be considered.

Position dependent dyspnea and hypoxemia.

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: Two Case Reports.

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. In the first case, a transesophageal echocardiogram (TEE) showed interatrial communication, ostium secundum type, with bidirectional shunting. Patient underwent a successful percutaneous closure of communication, with no residual shunting and clinical improvement and no positional hypoxemia. In the second case, infectious complications were the cause of hemodynamic changes producing meaningful right-to-left pressure gradients, resulting in POS. After antibiotic treatment there was a major clinical improvement and a second TEE showed bidirectional shunting with no positional variation. It was assumed resolution of POS after treatment of infectious complications with no need for immediate surgery. These two cases, with very distinctive functional and anatomic components, illustrate the challenge of understanding the exact mechanism by which POS results in clinical symptoms. A suggestive history and positional variation of oxygen saturation are very useful clues for its diagnosis in cases of unexplained hypoxemia.

Platypnea Orthodeoxia Syndrome Secondary to a Persistent Eustachian Valve.

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 patient had a complete resolution of her platypnea following the closure of the patent foramen ovale.

Coexistence of right-to-left and left-to-right shunts across patent foramen ovale and coronary cameral fistulas.

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.

Usefulness of a Pulse Oximeter and Multimodality Imaging for Diagnosing Platypnea-orthodeoxia Syndrome.

Platypnea-orthodeoxia syndrome (POS) is a rare disease characterized by dyspnea and hypoxemia in orthostatism that improves in the recumbent position. We herein report an 81-year-old woman with dyspnea in the upright position following thoracic vertebral compression fractures. After the patient's daughter brought a recording showing decreasing SpO2 (peripheral capillary oxygen saturation) in the upright position as measured by a portable pulse oximeter outside the hospital, a small atrial septal defect (ASD) was detected. A contrast echocardiogram and four-dimensional flow magnetic resonance imaging demonstrated a right-to-left shunt. The patient's symptoms dramatically improved after percutaneous ASD closure. In conclusion, such new technologies are useful for diagnosing POS.

Platypnea-orthodeoxia syndrome: A dangerous detour of intermediate syndrome with organophosphorus poisoning.

Dyspnea experienced in the upright position which is relieved in the supine position, is known as Platypnea. Accompanying desaturation in the arterial blood associated with Platypnea is known as orthodeoxia. Various causes of Platypnea orthodeoxia syndrome, such as hepatopulmonary syndrome, patent foramen ovale and atrial septal defects, are well described in the literature. However, a rather uncommon and underreported cause of Platypnea orthodeoxia syndrome is organophosphorus poisoning. We report a case series of three cases that were reported after ingestion of organophosphorus compounds and developed breathlessness later in the course of the hospital stay, which was diagnosed as Platypnea orthodeoxia syndrome resulting from organophosphorus poisoning. All three cases were treated with supportive management, incentive spirometry and respiratory physiotherapy and responded well to the treatment.

Cardiac device-induced right to left shunt causing significant hypoxemia: A case report and review of literature.

We describe a patient where a pacemaker lead induced tricuspid valve changes that caused a right to left shunt through a preexisting patent foramen ovale resulting in significant hypoxemia. This event occurred years after the pacemaker had been placed. Surgical closure of the patent foramen ovale resolved the patient's hypoxemia and dyspnea. We also reviewed the previous cases published in the medical literature concerning significant hypoxemia from cardiac device-induced right to left shunts. Three of the four reported cased occurred 4 or more years after device placement. Therefore, late onset hypoxemia in setting of cardiac device placement without an alternative cause should raise the possibility of this complication.

Platypnea Orthodeoxia for Those Who do Better Lying Low.

Platypnea orthodeoxia syndrome (POS) can be a confounding disease. Patients with POS may have already had an extensive and unrevealing evaluation for hypoxia. POS is the worsening of hypoxia when upright compared to supine. The underlying mechanism is a right to left shunt. While there are various causes of this, we focus on intracardiac shunt. The most common of these is patent foramen ovale (PFO). Once this is identified, closure of the PFO can lead to resolution of hypoxia.

Platypnea-Orthodeoxia Syndrome in the Setting of Patent Foramen Ovale Without Pulmonary Hypertension or Major Lung Disease.

Background Patent foramen ovale (PFO)-associated platypnea-orthodeoxia syndrome is characterized by dyspnea and hypoxemia when upright. The pathogenesis is thought to involve an increase in right atrial pressure or change in degree of right to left shunting with upright posture. Methods and Results We sought to characterize patients with platypnea-orthodeoxia syndrome related to PFO without pulmonary hypertension. We retrospectively reviewed databases at 3 tertiary referral hospitals in New South Wales, Australia from 2000 to 2019. Fourteen patients with a mean age of 69±14 years had a PFO with wide tunnel separation. Mean New York Heart Association Classification was II (±0.9) and 7 inpatients had been confined to bed (from postural symptoms). Baseline oxygen saturations supine were 93%±5% and 84%±6% upright. Two patients had a minor congenital heart defect and 4 had mild parenchymal lung disease with preserved lung function. The mean aortic root diameter was 37±6 mm and distance between aortic root and posterior atrial wall was 16±2 mm. Platypnea-orthodeoxia syndrome was preceded by surgery in 5 patients and 1 patient had mild pneumonia. Successful closure of the PFO using an Amplatzer device was performed in 11 of 14 patients. Post-closure, all patients had New York Heart Association Classification I (improvement 1.6±0.9, P<0.003) and semi-recumbent oxygen saturations increased by 13%±8% (P<0.001, n=10). Conclusions Platypnea-orthodeoxia syndrome is a debilitating condition, curable by PFO closure. Anatomical distortion of the atrial septum related to a dilated aortic root or shortening of the distance between the aortic root and posterior atrial wall may contribute to the syndrome.

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

A 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.