Platypnea-Orthodeoxia Syndrome Associated with Spontaneously Ruptured Chordae Tendineae of Tricuspid Valve.
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ABSTRACT:
SUBMITTER: Uchihashi M
PROVIDER: S-EPMC7175809 | biostudies-literature | 2020 Apr
REPOSITORIES: biostudies-literature
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Project description:We present the appearance of chordae tendineae calcification on transthoracic echocardiography and ECG-gated cardiac computed tomography in a 75 year-old woman. While the etiology is unclear, the abnormality can be clearly delineated on a properly performed CT study. We also discuss modification of the cardiac CT protocol to optimize visualization of the tricuspid valve apparatus.
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Project description:BackgroundParachute mitral valve with reticular chordae tendineae is an extremely rare anomaly.Case presentationWe present a case of parachute mitral valve associated with distinctive reticular chordae tendineae in an adult. It was diagnosed from the echocardiogram. The patient was referred for surgery. Valve analysis showed thickened mitral valve leaflets and commissures. The chordae tendinae were lengthy and thick. All the chordae tendinae merged into a solitary papillary muscle. A distinctive reticular fibrous tissue was found on mitral valve apparatus as the chordae tendinae intermixed each other. The only functional communication between the left atrium and the left ventricle was through the reticular spaces. This anomaly was considered to be unrepairable and was replaced with a mechanical valve.ConclusionsAn extremely rare and unique case of parachute mitral valve associated with reticular chordae tendineae was reported. Mitral valve replacement is a reasonable choice in patients with parachute mitral valve with reticular chordae tendineae.
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Project description:Platypnea-orthodeoxia syndrome, a rare condition characterized by posture-related dyspnea, is usually caused by an intracardiac shunt, hepatopulmonary syndrome, or shunting resulting from severe pulmonary disease. We report the case of a 33-year-old woman who presented with increasing dyspnea and oxygen desaturation when she sat up or arose. Our diagnosis was platypnea-orthodeoxia syndrome. A lead of a previously implanted pacemaker exacerbated a severe tricuspid regurgitant jet that was directed toward the patient's intra-atrial septum. Percutaneous closure of a small secundum atrial septal defect eliminated right-to-left shunting and substantially improved the patient's functional status. In addition to this case, we discuss this unusual condition.
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Project description:Platypnea orthodeoxia syndrome (POS) occurs when an upright position results in acute-onset hypoxemia and is relieved with recumbency. POS can be due to intracardiac shunting, intrapulmonary shunting, ventilation-perfusion mismatch, or a combination of these. We report a case of POS that developed 3 days post liver transplantation as a result of new-onset right to left shunting across a patent foramen ovale. Right heart catheterization revealed a posteriorly directed inferior vena cava likely due to altered inferior vena cava-right atrial junction anatomy as a result of liver transplantation. The patient underwent successful transcatheter patent foramen ovale closure with a 25-mm Gore Cardioform septal occluder device with immediate and sustained improvement in hypoxia.
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Project description:Platypnea-orthodeoxia syndrome (POS) is a rare condition characterized by dyspnoea and deoxygenation in an upright position that is relieved by supine positioning. There are only five published accounts of it occurring post-lobectomy. We present the case of a 72-year-old male with 3 months of supposedly unexplained dyspnoea after right lower lobectomy for lung cancer who was confirmed to have POS. We highlight the importance of recognition and management as well as provide a brief summary of the pathophysiology.
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Project description:Aims: This study aimed to investigate the pathology, classification, diagnosis, and surgical prognosis of UCMV. Methods and Results: Consecutive paediatric patients with ≥ moderate-severe mitral regurgitation (MR) and mitral stenosis (MS) were recruited between October 2016 and July 2020. UCMV was diagnosed and classified into three grades according to the involvement of chorda groups and MS presence or absence; other mitral lesions were included as controls. Of 207 eligible patients, 75 with UCMV (10.0 m [interquartile range (IQR): 6.0-21.5]) and 110 with other mitral lesions (16.0 m [IQR: 5.0-43.5]) were diagnosed using echocardiography and surgical exploration. The associated chorda groups of UCMV were confirmed to show high agreement between echocardiography and surgery (kappa = 0.857, p < 0.001). At baseline surgery assessment, the UCMV group exhibited worse New York Heart Association functional class, more severe MR and MS grades, and fewer associated complex anomalies (all, p < 0.05) than the control group. After a mean follow-up of 8.3 (IQR:2.7-14.4) months and adjustment for covariates, the UCMV group required longer cardiopulmonary bypass and aortic clamp times, but there were no differences in the incidence of adverse events (p = 0.584). Class III was associated with higher risk of adverse events than classes I and II (p = 0.002). Conclusions: The UCMV spectrum constitutes a primary pathogenesis of paediatric MV dysfunction, which can be optimally diagnosed using echocardiography. Classification based on mitral anatomy and dysfunction can predict the risk of postoperative adverse events.
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Project description:Platypnea-orthodeoxia syndrome is a rare disease defined by dyspnea and deoxygenation, induced by an upright position, and relieved by recumbency. Causes include shunting through a patent foramen ovale and pulmonary arteriovenous malformations. A 79-year-old woman experienced 2 syncopal episodes at rest and presented at another hospital. In the emergency department, she was hypoxic, needing 6 L/min of oxygen. Her chest radiograph showed nothing unusual. Transthoracic echocardiograms with saline microcavitation evaluation were mildly positive early after agitated-saline administration, suggesting intracardiac shunting. She was then transferred to our center. Right-sided heart catheterization revealed no oximetric evidence of intracardiac shunting while the patient was supine and had a low right atrial pressure. However, her oxygen saturation dropped to 78% when she sat up. Repeat transthoracic echocardiography while sitting revealed a dramatically positive early saline microcavitation-uptake into the left side of the heart. Transesophageal echocardiograms showed a patent foramen ovale, with right-to-left shunting highly dependent upon body position. The patient underwent successful percutaneous patent foramen ovale closure, and her oxygen supplementation was suspended. In patients with unexplained or transient hypoxemia in which a cardiac cause is suspected, it is important to evaluate shunting in both the recumbent and upright positions. In this syndrome, elevated right atrial pressure is not necessary for significant right-to-left shunting. Percutaneous closure, if feasible, is first-line therapy in these patients.
| S-EPMC4894711 | biostudies-literature
Project description:Background:Platypnea-orthodeoxia syndrome is a rare condition characterized by onset or worsening of dyspnoea with orthostatism (platypnea) and arterial desaturation (orthodeoxy) that is relieved by returning to a recumbent position. An acute event causing a change in the pressure relationships inside the cardiac chambers can lead to the diagnosis of a previously undiagnosed cardiac anomaly, as the following case illustrates. Case summary:A previously asymptomatic 80-year-old female patient was admitted in our hospital with a sudden onset ischaemic stroke. Initial evaluation, including 12-lead electrocardiogram and transthoracic echocardiogram, was unremarkable. During hospital stay, she develops pulmonary embolism, after which she complains of positional dyspnoea that develops upon sitting up, accompanied with refractory hypoxaemia that reverts on recumbent position. Transoesophageal echocardiogram revealed an interatrial septum with an exuberant hyperdynamic movement, and an abundant passage of contrast from the right atrium to the left, even without performing the Valsalva manoeuvre, compatible with an important patent foramen ovale. A percutaneous closure was performed, and patient has been symptom-free since then. Discussion:This is a case illustrates how an anomaly that has been present for 80?years can suddenly manifest itself with an array of different symptoms that can make the diagnosis challenging. A high degree of clinical suspicion is crucial for an accurate diagnosis and definitive treatment.
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Project description:We describe a case of platypnea-orthodeoxia syndrome (POS) due to atrial septal defect (ASD) occurring in the early postoperative course of a right pneumonectomy. Deformation of the atrial septum after right pneumonectomy deviates the blood from the inferior vena cava to ASD during the sitting position creating, a massive right-to-left shunt. Diagnosis can initially be missed by making contrast bubble test through the superior vena cava. The atrial septal defect was then closed using the surgical technique, allowing an instantaneous improvement of hematosis.
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