Platypnea-orthodeoxia Syndrome Research Articles

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

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

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 - Sequential Comparison of the Ascending Aorta's Anteversion to the Right Atrium.

Platypnea-Orthodeoxia Syndrome - Sequential Comparison of the Ascending Aorta's Anteversion to the Right Atrium.

6302018 Platypnea-orthodeoxia syndrome in pregnancy

6302018 Platypnea-orthodeoxia syndrome in pregnancy

Intraoperative Hypoxemia in the Right Lateral Decubitus Position: A Case Report

Platypnea-Orthodeoxia syndrome (POS), an exceptionally uncommon condition, is characterized by decreased oxygen saturation in an upright posture with corresponding improvement in the supine position. Recently, an 85-year-old female patient presented for surgery due to a left femoral neck fracture. Following successful tracheal intubation and anesthesia induction, the ventilator indicated an airway pressure of 15mmHg. While in the right lateral decubitus position, the patient developed hypoxia despite normal arterial blood pressure and symmetrical breath sounds. Attempts to alleviate the hypoxia through pure oxygen ventilation, tympanic lung ventilation, and high-frequency ventilation were unsuccessful. Subsequently, there was a notable decrease in both blood pressure and heart rate, prompting an immediate halt to the surgery and repositioning the patient to the supine position. This resulted in the normalization of the patient's heart rate, oxygen saturation, and blood pressure. To investigate the etiology, a TEE probe was inserted, revealing the presence of a patent foramen ovale (PFO) and a significant right-to-left shunt while the patient was in the lateral position. The procedure was performed in a specific right lateral decubitus position. This case may represent a unique manifestation of POS syndrome, expanding the differential diagnosis for intraoperative position-related hypoxemia. Given the severe hypoxemia observed with intraoperative positional changes, a strong suspicion of POS syndrome is warranted.

The paradox of platypnoea-orthodeoxia syndrome.

Platypnoea-orthodeoxia is a rare clinical syndrome characterised by dyspnoea and oxygen desaturation in the upright position which improves when supine. It requires two components: a sufficiently sized anatomical vascular defect (typically intra-cardiac or intra-pulmonary) combined with a functional component that promotes positional right-to-left shunting. We describe the rare occurrence of a patient with platypnoea-orthodeoxia syndrome (POS) because of a paradoxical shunt through a patent foramen ovale caused by a large right atrial line-associated thrombus in a male with metastatic oesophageal cancer undergoing chemotherapy. This case is a timely reminder to consider POS amongst differentials for hypoxia as it is often treatable if recognised.

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

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. 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. 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. 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.

Een ongebruikelijke oorzaak van acute desaturatie

An unusual cause of acute desaturation A 61-year-old man presented with symptoms of rapidly increasing dyspnea, chest pain and significant hypoxemia for which the initial investigations revealed no clear explanation. There was a remarkable improvement of the saturation in supine position. A contrast transesophageal echocardiography established the presence of a patent foramen ovale (PFO), which was successfully closed via a percutaneous transcatheter. Platypnoea-orthodeoxia syndrome (POS) is a rare entity characterized by the presence of arterial hypoxemia in upright position with resolution of the hypoxemia in supine position. It is most frequently caused by a PFO. A PFO is, however, merely an essential precondition and the development of POS nearly always requires the presence of other (functional) factors.

Patent Foramen Ovale Percutaneous Closure: Evolution and Ongoing Challenges.

Patent foramen ovale (PFO) concerns nearly a quarter of the general population and incidence may reach up to 50% in patients with cryptogenic stroke. Recent randomized clinical trials confirmed that percutaneous closure of PFO-related stroke reduces the risk of embolic event recurrence. PFO also comes into play in other pathogenic conditions, such as migraine, decompression sickness or platypnea-orthodeoxia syndrome, where the heterogeneity of patients is high and evidence for closure is less well-documented. In this review, we describe the current indications for PFO percutaneous closure and the remaining challenges, and try to provide future directions regarding the technique and its indications.

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.

"A Hole with a Heart Around: Platypnea-Orthodeoxia Syndrome"

"A Hole with a Heart Around: Platypnea-Orthodeoxia Syndrome"

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

Abstract 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 cause of positional hypoxaemia: platypnoea-orthodeoxia syndrome associated with patent foramen ovale and ascending aortic dilatation.

An unusual cause of positional hypoxaemia: platypnoea-orthodeoxia syndrome associated with patent foramen ovale and ascending aortic dilatation.

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.

Surgical repair of platypnea-orthodeoxia syndrome caused by patent foramen ovale, ascending aortic dilation, and pectus excavatum: a case report

BackgroundPlatypnea-orthodeoxia syndrome (POS) is a relatively uncommon clinical syndrome characterized by dyspnea and deoxygenation accompanying postural changes, such as sitting or standing from a recumbent position.Case presentationWe describe the case of a 76-year-old woman who was hospitalized with dyspnea in the sitting position, which was relieved in the right lateral decubitus position. The patient was diagnosed with POS caused by a patent foramen ovale (PFO), dilation and elongation of the ascending aorta, and pectus excavatum. We performed PFO closure and replacement of the ascending aorta via median sternotomy. The patient’s postoperative percutaneous oxygen saturation dramatically increased in the sitting position.ConclusionsElongated and dilated ascending aorta and pectus excavatum with PFO may be risk factors for POS. In older patients, surgical intervention is an important treatment option for POS caused by cardiac and aortic comorbidities.

Patent Foramen Ovale Closure in a Nonagenarian With Platypnoea-Orthodeoxia Syndrome

Patent Foramen Ovale Closure in a Nonagenarian With Platypnoea-Orthodeoxia Syndrome

Patent Foramen Ovale Closure in a Nonagenarian With Platypnoea-Orthodeoxia Syndrome

Patent foramen ovale (PFO) is a common congenital cardiac abnormality that affects one-quarter of the general population. Hypoxaemia, because of large right-to-left shunt, is a rare complication of PFO and occurs under exceptional physiological conditions. Case: This 90-year-old lady presented with progressive dyspnoea over several months. At presentation to hospital, she was hypoxaemic with pO2 of 53 mmHg and oxygen saturation of 89% despite 15L of oxygen via a mask. While lying supine for a computed tomography pulmonary angiogram (CTPA), it was noted that her oxygen saturation normalised. The CTPA excluded pulmonary embolus and intrapulmonary vascular anomaly. Intermittent intracardiac shunt was suspected and confirmed on formal shunt testing with a shunt fraction calculated at 32%. Transthoracic echocardiogram and cardiac CT failed to demonstrate an intracardiac shunt. Transoesophageal echocardiogram confirmed a PFO with dynamic right-to-left shunting on colour Doppler and with agitated saline. She underwent percutaneous closure with a 25-mm Amplatzer PFO Occluder, with a good procedural result and resolution of her symptoms. At the 2-month follow-up she remained well with no breathlessness and no device-related complications. This case illustrates an unusual cause of platypnoea-orthodeoxia syndrome due to PFO in a nonagenarian. It was hypothesised that a prominent Eustachian valve, and anatomical factors including enlarged tortuous aorta and pectus excavatum, had gradually progressed over time, distorting the cardiac anatomy, and contributing to significant shunting. Percutaneous PFO closure resulted in immediate symptomatic benefit.

P143 PLATYPNOEA–ORTHODEOXIA: A CASE REPORT OF A RARE SYNDROME

Abstract A 32–year–old non–smoker Asian female was admitted to the Emergency Department San Salvatore Hospital of L‘Aquila experiencing worsening of dyspnea. Three months earlier the patient had undergone left pneumonectomy. An arterial blood gas analysis performed on air reported: pH 7.53, pCO2 23.7 mmHg, pO2 38.7 mmHg, SpO2 80%. Blood tests were within normal limits, except for a moderate increase in D–Dimer. Considering the diagnostic hypothesis of pulmonary embolism, an Angio CT–scan of the chest was performed and showed no radiological characteristics suggesting parenchymal pathology and no evidence of thromboembolic or other vascular pathology. From the accurate clinical evaluation of the patient, a condition of severe platypnoea was evident: supine decubitus was obliged. Together with the above position variations, a strong desaturation was observed, going from almost normal values (94–96% SpO2), to 80–82% SpO2 when she was sitting up in bed. Based on the data above, a right–left shunt condition was assumed, and a transesophageal contrast echocardiogram was requested. The latter demonstrated patency of the foramen ovale. The first description of a condition very similar to platypnoea–orthodeoxia syndrome following pneumonectomy dates to 1956, by Schnabel et al. A recent pubmed database–EMBASE systematic review identified 8 studies concerning ten patients, 6 male/4 female. The mean age was 62 years. All patients underwent resection of the right lung. The time–interval between the operation and the platypnoea–orthodeoxia syndrome (POS) was from 2 days to 3 years. A PFO was present in 90%. Considering the data, our patient shows at least two peculiarities; the first and most relevant is the young age: in fact, there is only one description of POS in a patient of younger age. The second peculiarity is the left localization of the previous pneumonectomy, much rarer in frequency than the contralateral in the known cases. Partial or total pulmonary resection can rarely be followed by the onset of POS. This syndrome can be severely disabling, so physicians must be prepared for its prompt recognition, maintaining a high degree of suspicion.

C13 AT THE ROOT OF HYPOXIA: NEVER UNDERESTIMATE THE CLINICAL

Abstract A 74–year–old woman arrived to the ER for syncope, reporting episodes of atypical chest pain for a few months. The initial ECG showed negative T waves in the lateral leads; an echocardiogram documented lower–limit systolic function due to akinesia of the antero–lateral segments and the presence of an ascending aorta aneurysm (AAA). The woman, with multiple cardiovascular risk factors, had previous transmural myocardial ischemia documented by MRI and never investigated with coronary angiography (ICA). Admitted to ICCU for suspected ischemic heart disease, she experienced a sudden clinical deterioration with onset of acute respiratory failure, initially stabilized with noninvasive ventilation. A chest angioCT excluded acute events (pulmonary embolism, aortic dissection) and confirmed the presence of AAA with bovine arch. Given the persistent need for oxygen therapy, closer clinical observation allowed objectification of platypnea–orthodeoxia syndrome (POS) that presented with desaturation, dyspnea, and mental confusion in the orthostatic position that improved with supine position. A bubble test in the two positions documented worsening intracardiac shunt in the sitting position, and a transesophageal echocardiogram showed the presence of a tunnel–like patent foramen ovale (PFO). The patient then underwent ICA that found a stenosis–free vascular tree, but a marked fusiform common trunk aneurysm (CTA) was appreciated. After exclusion of altered cardiopulmonary hemodynamic balance, percutaneous closure of the PFO was performed, which allowed progressive improvement of respiratory exchanges and subsequent complete weaning from oxygen. Typically, PFO occurs in the case of a pulmonary ventilo–perfusory mismatch or right–left shunt (rlS), associating mainly with atrial congenital defects and, to a lesser extent, pulmonary arteriovenous malformations. Sometimes, for POS to occur, the presence of a functional component facilitating rlS is necessary. In our case, it is conceivable that such a contribution was both ab–extrinsic compression of the right atrium by the AAA and compression of the pulmonary trunk at the level of the pulmonary valve by the CTA. This diagnostic hypothesis is compatible with the late presentation.