Platypnea-orthodeoxia syndrome developed with the progression of pulmonary parenchymal involvement: A case report.

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 objective: Platypnea-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.

Platypnea-orthodeoxia syndrome in a patient with poor activity of daily living: Struggling with a definitive diagnosis.

A 73-year-old female patient was diagnosed with lumbar spinal stenosis by an orthopedic surgeon. During admission for further evaluation, she was found to have hypoxemia. Contrast-enhanced computed tomography revealed a 43-mm ascending aortic aneurysm, but there were no signs of pulmonary embolism, and no abnormalities were detected in the lung fields. Upon initiating rehabilitation in the standing position, respiratory distress and hypoxemia worsened. Careful observation revealed that hypoxemia worsened in the seated position but normalized while lying down. We considered the possibility of platypnea-orthodeoxia syndrome (POS), in which hypoxemia worsens in the seated position. Transesophageal echocardiography revealed that a patent foramen ovale (PFO), which was hardly noticeable while lying down, worsened in the seated position. A pulmonary perfusion scan showed a 27 % right-to-left shunt. Cardiac catheterization confirmed the presence of right-to-left shunting during right atrial injection. Consequently, it was diagnosed that the ascending aortic aneurysm aggravated the PFO in the seated position, leading to POS. The PFO was unsuitable for transcatheter treatment. Consequently, the patient underwent direct closure surgery in the cardiac surgery department. Postoperatively, the patient's hypoxemia and respiratory distress in the seated position improved, and subsequent progress has been favorable. Learning objective: Diagnosing platypnea-orthodeoxia syndrome in patients with poor activities of daily living (ADL) is challenging. Careful observation of the percutaneous oxygen saturation in both supine and seated positions is crucial, and a transesophageal echocardiogram in the supine and seated positions is inevitable. Lung perfusion scintigraphy is often used to evaluate the cause of hypoxemia; however, whole-body scans are important for detecting the presence and number of right-left shunts. This case report highlights the pitfalls of diagnosis in patients with poor ADL.

Platypnea-Orthodeoxia Syndrome After the Bentall Procedure.

Platypnea-orthodoxia syndrome (POS) is a clinical condition that causes dyspnea and hypoxia in the sitting and standing positions. In this case, a 67-year-old man showed hypoxemia after undergoing the Bentall procedure that worsened in the standing position during rehabilitation. Contrast-enhanced computed tomography of the thorax and abdomen revealed no cause of respiratory failure. POS was suspected as the cause of the positional exacerbation of oxygen saturation. A bubble study showed a positive grade IV within three heartbeats on transthoracic echocardiography, which also confirmed an intracardiac shunt caused by a patent foramen ovale (PFO). Percutaneous PFO closure was performed, and hypoxemia was immediately resolved. Various factors were considered to cause the POS, including right heart failure, constrictive pericarditis, and postoperative adhesions, and each of these factors was discussed. POS after open-heart surgery is very rare. This is the first reported case of POS treated with a closure device following the Bentall procedure.

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.

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.

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.

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.

Sinus venosus atrial septal defect presenting with platypnea-orthodeoxia syndrome in the setting of COVID-19 infection.

Platypnea-orthodeoxia syndrome is characterised by hypoxemia and dyspnoea while upright that resolves when supine, typically associated with an interatrial communication. We present a case of platypnea-orthodeoxia syndrome associated with a sinus venosus atrial septal defect in a patient with multiple possible aetiologies of hypoxemia, including COVID-19 infection. Cardiac catheterisation with provocative maneuvers confirmed the diagnosis and symptoms resolved following defect closure. We suggest that in patients with platypnea-orthodeoxia syndrome, it is useful to obtain haemodynamic data while supine and upright.

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.

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.

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.

An unusual approach for platypnea-orthodeoxia syndrome due to isolated pulmonary arteriovenous malformations.

A 27-year-old man presented to the emergency department with complaints of syncope, dyspnea, and fatigue. Physical examination revealed left-sided mild hemiparesis, platypnea, and continuous murmur on right middle lobe lung auscultation.

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.

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.

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.

Hypoxia Due to a Large Right to Left Interatrial Shunt with Normal Right-Sided Filling Pressures in the Setting of a Separation between the Septum Primum and Secundum: A Case Report.

Hypoxia secondary to right to left interatrial shunting in the setting of normal right-sided filling pressure is an uncommon clinical presentation. We present a case of persistent hypoxia irrespective of body position in a patient with a wide separation between the septum primum and secundum, creating a tunnel channeling flow from the right to the left atrium. Hypoxia resolved instantly following closure of the defect with an Amplatzer occluder under intracardiac echocardiography guidance. Although platypnea-orthodeoxia leads to hypoxia in the setting of normal right-sided filling pressures, the finding of a coexisting wide gap between the septum primum and secundum is likely to have worsened the hypoxia making it severe irrespective of body position. Closure of the interatrial septal defect led to immediate and sustained resolution of the hypoxia.

Managing Congenital Heart Defects in Elderly: The Platypnea-Orthodeoxia Syndrome in Underestimated Patent Foramen Ovale.

The platypnea-orthodeoxia syndrome (POS) is a rare and often suboptimally managed condition with a complex diagnostic workup, conversely displaying an easy treatment and a good recovery of symptoms, especially if consequent to an intracardiac shunt. However, its identification is challenging, due to the several clinical manifestations, the multiple etiologies, representing often the delayed presentation of a congenital heart disease. We present a case report and review of available literature on patients with the POS secondary to a patent foramen ovale successfully treated with its closure.

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.