Cor triatriatum dexter in children: Literature review and case report.
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SUBMITTER: Kalangos A
PROVIDER: S-EPMC8304494 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:BackgroundTricuspid regurgitation is gaining importance due to its high morbidity and mortality. Especially in the elderly, novel technologies in percutaneous therapies have become valuable options due to the commonly present high surgical risk.Case presentationWe report a case of a 78-year-old female suffering from massive tricuspid regurgitation with repetitive right-sided heart failure hospitalizations. As the patient was very frail and deemed as high surgical risk, we used the TriClip® system to improve her symptomatic status. During diagnostic work-up, an additional membrane separating the right atrium, consistent with the definition of a cor triatriatum dexter, was found. Although increasing the complexity of the procedure, implantation of 3 clips with reduction of tricuspid regurgitation to a mild-to-moderate degree was achieved without any notable complications. The patient was discharged with ameliorated symptoms on the fourth postoperative day.ConclusionsOur case highlights the feasibility of percutaneous edge-to-edge tricuspid valve repair in an elderly woman with cor triatriatum dexter. Accurate echocardiographic visualization is an absolute requirement to gain access to the tricuspid valve without interacting with prevailing additional membranes.
| S-EPMC8467593 | biostudies-literature
Project description:Percutaneous balloon dilation was performed in a Rhodesian Ridgeback and in an American Staffordshire Terrier affected by cor triatriatum dexter (CTD). Both cases had ascites without jugular venous distension or pleural effusion. In both dogs the CTD presented a perforated membrane but with different morphology: in one case the coronary sinus entered the caudal chamber of the CTD together with the caudal vena cava. In the other case, the coronary sinus communicated with the cranial chamber of the CTD together with the cranial vena cava. Percutaneous balloon dilation of the CTD was successfully performed, and both dogs had uneventful surgery recoveries. At two years of follow-up, the dogs were free from clinical signs and cardiac medication.
| S-EPMC9412336 | biostudies-literature
Project description:Cor-triatriatum dexter is an extremely rare congenital heart defect in which there is complete persistence of the right valve of embryonic sinus venosus that results in partitioning of the right atrium into a smooth and trabeculated portion. The smooth portion receives venous blood from inferior vena cava, superior vena cava, and coronary sinus while the trabeculated portion contains the right atrial appendage and the opening of tricuspid valve. We report a 1-week-old child who presented with intermittent episodes of central cyanosis. Echocardiography, established, and bubble contrast study confirmed the diagnosis of an isolated cor-triatriatum dexter. The baby initially underwent an intervention by cardiac catheterization, which was unsuccessful in disrupting the membrane and re-direct the systemic venous flow to the right heart chambers. She subsequently had the cor-triatriatum dexter membrane resected via an uncomplicated open-heart surgery.
| S-EPMC4782468 | biostudies-literature
Project description:Cor triatriatum dexter is an extremely rare congenital heart defect and cause of hypoxia in adults. We describe a case of cor triatriatum dexter discovered incidentally due to an iatrogenic atrial septal defect. The cor triatriatum dexter resolved with balloon dilation - a novel technique to manage this rare clinical condition. <Learning objective: Management of cor triatriatum dexter, an exceptionally rare diagnosis, has often been thought to be surgical. However, percutaneous intervention may be considered in select clinical situations.>.
| S-EPMC8766317 | biostudies-literature
Project description: Not available
| S-EPMC6833460 | biostudies-literature
Project description:BackgroundCor triatriatum sinister (CTS) is a rare congenital cardiac anomaly defined by a fibromuscular membrane which bisects the left atrium. Cor triatriatum sinister has been associated with cardioembolic stroke through mechanisms including stagnation of blood flow within the left atrium, an association with atrial fibrillation (AF), and/or an accompanying atrial septal defect (ASD) or patent foramen ovale. We describe a case highlighting the role that CTS may play in cardioembolic stroke, provide high-quality computed tomography angiography and two- and three-dimensional echocardiography of the CTS membrane, and outline management strategies for this uncommon clinical scenario.Case summaryA 35-year-old man with no prior medical history presented with acute onset weakness and aphasia. He was found to have an embolic stroke with left M1 and A1 occlusions and received tissue plasminogen activator followed by mechanical thrombectomy with successful recanalization. A thorough stroke workup revealed CTS with an associated ASD as well as potential protein C deficiency. He was managed with indefinite anticoagulation with apixaban.DiscussionThis is the 13th reported case of CTS associated with stroke. In most previous cases evidence of blood stasis or frank thrombus was associated with the CTS membrane, and/or existing AF was noted. In this case, none of these were identified, particularly highlighting the surreptitious risk of CTS. In addition, the presence of potential protein C deficiency in this case compounded the risk for thromboembolism and factored into multidisciplinary management decisions.
| S-EPMC7319846 | biostudies-literature
Project description:BackgroundCor triatriatum sinistra (CTS) is a rare condition where the left atrium (LA) is divided by a thin membrane into an upper and lower chamber. Incidentally, the diagnosis is made in late adulthood, usually because of a favourable variant such as in our patient who presented with partial CTS.Case summaryWe present the case of a 62-year-old female who presented with COVID-19. She was known for longstanding symptoms of dyspnoea on exertion as well as a minor stroke several years ago. Computed tomography on admission suggested there was a mass in the LA but transthoracic echocardiography and cardiac magnetic resonance imaging revealed the diagnosis of partial CTS in which the superior compartment received pulmonary venous drainage from the right lung and the left-sided pulmonary veins drained into the inferior chamber. Since there were signs of chronic pulmonary oedema she successfully underwent balloon dilatation of the membrane resulting in remission of symptoms and normalization of the pressure in the accessory chamber.DiscussionPartial CTS is a rare variant of CTS. Since part of the pulmonary veins drains in the lower chamber of the LA (and thereby unload the right ventricle), it is a favourable variant and patients may present later in life when membrane orifices calcify or it may be discovered as an incidental finding. In some patients requiring intervention, balloon dilatation of the membrane may be considered as an alternative to surgical removal of the membrane by thoracotomy.
| S-EPMC10064265 | biostudies-literature
Project description:Bullous pemphigoid (BP) is a rare, life-threatening autoimmune blistering disease with pruritus and tension blisters/bullous as the main clinical manifestations. Glucocorticosteroids are the main therapeutic agents for it, but their efficacy is poor in some patients. Tofacitinib, a small molecule agent that inhibits JAK1/3, has shown incredible efficacy in a wide range of autoimmune diseases and maybe a new valuable treatment option for refractory BP. To report a case of refractory BP successfully treated with tofacitinib, then explore the underlying mechanism behind the treatment, and finally review similarities to other cases reported in the literature. Case report and literature review of published cases of successful BP treatment with JAK inhibitors. The case report describes a 73-year-old male with refractory BP that was successfully managed with the combination therapy of tofacitinib and low-dose glucocorticoids for 28 weeks. Immunohistochemistry and RNA sequencing were performed to analyze the underlying mechanism of tofacitinib therapy. A systematic literature search was conducted to identify other cases of treatment with JAK inhibitors. Throughout the 28-week treatment period, the patient experienced clinical, autoantibody and histologic resolution. Immunohistochemical analysis showed tofacitinib significantly decreased the pSTAT3 and pSTAT6 levels in the skin lesions of this patient. RNA sequencing and immunohistochemical testing of lesion samples from other BP patients identified activation of the JAK-STAT signaling pathway. Literature review revealed 17 previously reported cases of BP treated with four kinds of JAK inhibitors successfully, including tofacitinib (10), baricitinib (1), upadacitinib (3) and abrocitinib (3). Our findings support the potential of tofacitinib as a safe and effective treatment option for BP. Larger studies are underway to better understand this efficacy and safety.
2024-11-13 | GSE278926 | GEO
Project description: Not available
| S-EPMC5779145 | biostudies-literature
Project description:Cor triatriatum dexter (CTD) is an extremely uncommon and underreported congenital cardiac anomaly in which the persistence of the embryonic right venous valve separates the right atrium into two chambers with varying degrees of obstruction to antegrade flow and variable degree of right to left shunt at atrial level. Depending on the size of the valves, clinical manifestations vary from absence of symptoms to severe hypoxia, requiring urgent surgical correction. We herein describe the diagnostic difficulties in a case of neonatal CTD, who developed increasingly severe and unresponsive cyanosis, first interpreted as postnatal maladjustment with pulmonary hypertension. The failure to respond to oxygen and pulmonary vasodilators led us to reconsider a different diagnosis. The use of contrast echocardiography improved the diagnostic performance of transthoracic echocardiogram (TTE) and revealed a massive right-to-left shunt secondary to the presence of an atrial membrane that required urgent surgery.
| S-EPMC9139359 | biostudies-literature