Transcranial Doppler in the blue stander: the platypnoea-orthodeoxia syndrome revisited.
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ABSTRACT:
SUBMITTER: Egido JA
PROVIDER: S-EPMC4208255 | biostudies-literature | 2014 Oct
REPOSITORIES: biostudies-literature
Publications
Transcranial Doppler in the blue stander: the platypnoea-orthodeoxia syndrome revisited.
Egido Jose Antonio JA Garcia Ana Maria AM
BMJ case reports 20141020
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Project description:A 50-year-old man presented with dyspnoea while sitting, standing and walking but resolved completely in supine position. On cardiorespiratory examinations, fine crackles were noted over bibasal area. Chest X-ray showed bilateral reticulonodular shadows, restrictive pattern on spirometry, elevated alveolar arterial O2 gradient on arterial blood gas. High-resolution CT of the thorax revealed pattern as 'confident' or 'certain' radiographic diagnosis of idiopathic pulmonary fibrosis (IPF). Bubble-contrast echocardiography in recumbent, sitting and upright positions revealed no intracardiac (right to left shunt) or intrapulmonary shunts. This case highlights the necessity of awareness of this syndrome in cases of interstitial lung diseases (ILDs) also. Although 188 cases have been described thus far of platypnoea-orthodeoxia syndrome (P-OS) of various aetiologies, to the best of our knowledge, it is the first ever case of P-OS in ILD/IPF. Both lung bases were predominantly affected in this patient, platypnoea and orthodeoxia were attributed to areas of low/zero ventilation/perfusion (V/Q) ratio (zone 1 phenomena) as no other obvious explanation was found.
| S-EPMC3962980 | biostudies-literature
Project description:BackgroundPlatypnoea-orthodeoxia syndrome (POS) is a rare condition characterized by hypoxaemia and dyspnoea when changing from a recumbent to an upright position. Diagnosis requires a high clinical suspicion and is often underdiagnosed.Case summaryWe report a case of POS in a 50-year-old woman with dyspnoea and new-onset atrial fibrillation. Oxygen saturation and dyspnoea worsened as she changed from a supine to a sitting position (96 vs. 86%, respectively). Transoesophageal echocardiography demonstrated enlargement of both atria and right ventricle with reduced systolic function and a large Chiari network (CN). Colour Doppler discovered severe tricuspid regurgitation with tenting and tethering of the valve leaflets. Finally, a bubble test revealed the cause of POS to be a patent foramen ovale along with the severe tricuspid regurgitant jet moving into the left atrium and favoured by the CN. Surgical closure of the foramen ovale resulted in the resolution of symptoms.DiscussionPlatypnoea-orthodeoxia syndrome is most commonly caused by a right-to-left shunt through an anatomical defect of the interatrial septum, typically a patent foramen ovale, combined with elevated right atrium pressure. This case illustrates an uncommon cause of POS in the absence of elevated atrium pressure due to the interplay of three key elements: a patent foramen ovale, tricuspid regurgitation, and the CN. Our aim is to alert physicians to the possibility of an intracardiac shunt as the cause of unexplained and/or refractory hypoxaemia related to position changes. Early recognition of this syndrome promotes timely treatment, greatly improving patient outcomes.
| S-EPMC8453386 | biostudies-literature
Project description:BackgroundPlatypnoea-orthodeoxia syndrome (POS) is an uncommon condition characterized by dyspnoea and arterial desaturation in the standing or sitting position that improves in the supine position.Case summaryWe report two cases of POS caused by an atrial septal defect (ASD) and a patent foramen ovale (PFO). Both cases reported a recent decrease in body weight of more than 10 kg in a short time period. Transoesophageal echocardiography (TOE) with agitated saline bubble study revealed and a large amount of contrast bubble through the ASD (Patient 1) or the PFO (Patient 2) from the right atrium to the left atrium in the sitting position. Both patients were diagnosed by the finding of positional dyspnoea and the results of TOE using agitated saline bubble contrast.DiscussionTaken together, their presentations suggest that weight loss in a short time period could be a pathogenic factor for POS.
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Project description: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.
| S-EPMC9321992 | biostudies-literature
Project description:Platypnoea orthodeoxia is a rare clinical syndrome characterised by dyspnoea and deoxygenation exhibited in upright position with improvement in supine posture. Previously described aetiologies include cardiac (pericardial effusion or constrictive pericarditis), pulmonary (pneumonectomy, emphysema or amiodarone toxicity), abdominal (cirrhosis or ileus) or vascular (abdominal aortic aneurysm) causes. We report an unusual case of platypnoea orthodeoxia associated with patent foramen ovale and partial hepatic resection.
| S-EPMC4009827 | biostudies-other
Project description:BackgroundRupture of sinus of Valsalva (RSV) to right atrium (RA) causes significant left to right shunt, tricuspid regurgitation, and right ventricular failure. If left uncorrected it can lead to biventricular heart failure. Hence, early invasive management is advised. To date, there is no report about platypnoea-orthodeoxia syndrome (POS) after device closure of ruptured sinus of Valsalva.Case summaryA 50-year-old woman with dyspnoea of exertion and rupture of sinus valsalva to right atrium was referred to our hospital. On admission, chest computed tomography (CT) was normal. After closure of the rupture, she developed orthostatic hypoxemia and frequent cough. A repeat chest CT was suggestive of COVID-19 infection which most probably occurred during the hospitalization. Although COVID-19 was thought to be the only culprit, her symptoms were not solely justified by COVID-19. Transthoracic echocardiography showed patent foramen ovale (PFO) with significant shunt. PFO device closure was performed under intracardiac echocardiography guidance.DiscussionInteratrial septum deformation may happen after RSV correction and right to left shunt from PFO may become more significant. POS is an important indication for PFO closure which should be noticed by careful examination. As COVID-19 is the most frequent pathology these days, it may delay other probable diagnosis, and hence detailed history taking and physical examination is mandatory.
| S-EPMC8184264 | biostudies-literature
Project description:BackgroundPlatypnoea-orthodeoxia syndrome (POS) is characterized by dyspnoea and arterial desaturation in the sitting position. Although its pathophysiology is complex and still needed to be investigated, the disease is one of the clinical situations which should be immediately and adequately managed by health care workers from the initial presentation.Case summaryA 66-year-old woman with a history of systemic lupus erythematosus, deep vein thrombosis, and lumbar compression fracture was admitted for evaluation of the sudden onset of dyspnoea, while in the sitting position that was relieved on placing her in the supine position. Her transoesophageal echocardiogram did reveal a deformity in the patent foramen ovale (PFO) structure with a wide gap due to aortic compression, which was markedly different from that observed in the supine position, along with massive right-to-left shunting caused by redirected venous return due to a persistent Eustachian valve. With the computed tomography and angiograms, POS was diagnosed. Then, the patient received aortic replacement and patch closure of PFO, and her symptoms were completely resolved.DiscussionPlatypnoea-orthodeoxia syndrome is a condition with quite unique features and needs multiple clinical measures for the diagnosis and medical management. For all health care workers, it is essential to have a high suspicion in order to detect POS in patients with unexplained dyspnoea. Echocardiography plays a major role in establishing the diagnosis and offering the choice of therapeutic options.
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Project description:BackgroundThe reproducibility of transcranial Doppler (TCD) ultrasound measurements in Sturge-Weber syndrome (SWS) and TCD's ability to predict neurological progression is unknown.MethodsIn 14 individuals with SWS, TCD measured mean flow velocity, pulsatility index, peak systolic velocity, and end-diastolic velocity in the middle, posterior, and anterior cerebral arteries of the affected and unaffected hemisphere. TCD was performed either once (n = 5) or twice in one day (n = 9). We assessed the reproducibility of the measurements performed twice on the same day on subjects and compared the TCD measurements to previously published age-matched controls. Clinically obtained neuroimaging was scored for extent and severity of SWS brain involvement. Patients were prospectively assigned SWS neuroscores.ResultsMiddle cerebral artery velocity (r = 0.79, P = 0.04, n = 7), posterior cerebral artery velocity (r = 0.90, P = 0.04, n = 5), and anterior cerebral artery pulsatility index (r = 0.82, P = 0.02, n = 7) were reproducible TCD measurements comparing same-day percent side-to-side differences. In subjects with SWS, affected and unaffected mean peak systolic velocity and end-diastolic velocity in the middle, posterior, and anterior cerebral arteries were globally lower compared with age-matched control subjects. Subjects with the lowest affected middle cerebral artery velocity had the greatest worsening in the total neurological score between time 1 and 2 (r = -0.73, P = 0.04, n = 8) and the most severe magnetic resonance imaging involvement of the affected frontal lobe (r = -0.82, P = 0.007, n = 9).ConclusionsTCD may be a reliable measure with potential clinical value, indicating that blood flow may be globally decreased in SWS patients with unilateral brain involvement.
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Project description: Not available
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Project description:Post-concussion syndrome (PCS) refers to a constellation of physical, cognitive, and emotional symptoms after traumatic brain injury (TBI). Despite its incidence and impact, the underlying mechanisms of PCS are unclear. We hypothesized that impaired cerebral autoregulation (CA) is a contributor. In this article, we present our protocol for non-invasively assessing CA in patients with TBI and PCS in a real-world clinical setting. A prospective, observational study was integrated into outpatient clinics at a tertiary neurosurgical center. Data points included: demographics, symptom profile (Post-Concussion Symptom Scale [PCSS]) and neuropsychological assessment (Cambridge Neuropsychological Test Automated-Battery [CANTAB]). Cerebrovascular metrics (nMxa co-efficient and the transient hyperaemic-response ratio [THRR]) were collected using transcranial Doppler (TCD), finger plethysmography, and bespoke software (ICM+). Twelve participants were initially recruited but 2 were excluded after unsuccessful insonation of the middle cerebral artery (MCA); 10 participants (5 patients with TBI, 5 healthy controls) were included in the analysis (median age 26.5 years, male to female ratio: 7:3). Median PCSS scores were 6/126 for the TBI patient sub-groups. Median CANTAB percentiles were 78 (healthy controls) and 25 (TBI). nMxa was calculated for 90% of included patients, whereas THRR was calculated for 50%. Median study time was 127.5 min and feedback (n = 6) highlighted the perceived acceptability of the study. This pilot study has demonstrated a reproducible assessment of PCS and CA metrics (non-invasively) in a real-world setting. This protocol is feasible and is acceptable to participants. By scaling this methodology, we hope to test whether CA changes are correlated with symptomatic PCS in patients post-TBI.
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