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| http://dx.doi.org/10.1136/hrt.22.1.94 | DOI Listing |
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Ebstein's anomaly in children and young adults: clinical features, arrhythmia, surgical management, and factors affecting arrhythmia and mortality.
Cardiol Young
January 2025
Department of Cardiovascular Surgery, Hacettepe University, Ankara, Turkey.
Background: Ebstein's anomaly represents 40% of congenital tricuspid valve abnormalities. Studies about paediatric Ebstein's anomaly patients are limited.
Aim: To evaluate clinical characteristics, treatment (medical/arrhythmia ablation/surgical) results, and outcome of Ebstein's anomaly patients, and to determine factors affecting arrhythmia presence and mortality.
Intraoperative Hemodynamic Instability in a Patient With Ebstein's Anomaly Complicated With Eisenmenger Syndrome.
Case Rep Cardiol
December 2024
Department of Anesthesiology and Pain Management, Cleveland Clinic, Cleveland, Ohio, USA.
Ebstein's anomaly is a rare congenital displacement of the tricuspid valve resulting in atrialization of the right ventricle. About half of the patients with Ebstein's anomaly also have atrial septal defects, which may lead to chronic shunting and development of Eisenmenger syndrome. We describe a case of a sexagenarian male patient with a history of Ebstein's anomaly complicated with Eisenmenger syndrome undergoing robotic laparoscopic adrenalectomy who presented hemodynamic instability, hypoxemia, and likely right-to-left shunting intraoperatively, as well as the actions taken to correct it and have a successful outcome.
View Article and Find Full Text PDFComplete surgical correction of tetralogy of Fallot with Ebstein's anomaly.
Ann Pediatr Cardiol
November 2024
Department of CVTS, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Association of Ebstein's anomaly of tricuspid valve (TV) with tetralogy of Fallot (TOF) is extremely rare. Their coexistence is unique as they modify each other's physiology. We report a case of a girl child having this rare combination along with Diamond-Blackfan syndrome awaiting a bone marrow transplant.
View Article and Find Full Text PDFTwin atrioventricular nodes and accessory pathways in congenital heart diseases with abnormal atrioventricular connections: Association with the developmental hierarchy of cardiac morphology.
Heart Rhythm
November 2024
Department of Pediatrics, National Taiwan University Hospital and Medical College, National Taiwan University, Taipei, Taiwan.
Background: Twin atrioventricular (AV) nodes (TWAVNs) are common in heterotaxy syndrome.
Objective: The purpose of this study was to investigate the presence and implications of TWAVNs and accessory pathways in congenital heart diseases (CHDs) with abnormal AV connections.
Methods: A retrospective study of a 1980-2022 cohort with sufficient electrocardiographic (ECG) data for review was conducted.
Rapid identification of primary atopic disorders (PAD) by a clinical landmark-guided, upfront use of genomic sequencing.
Allergol Select
October 2024
Center for Child and Adolescent Health, Helios Hospital Krefeld, Academic Hospital of RWTH Aachen, Krefeld.
Article Synopsis
- Primary atopic disorders (PAD) are rare genetic conditions caused by specific gene variants that affect skin and immune function, making diagnosis challenging among common allergic disease cases.
- Identifying PAD requires recognizing clinical red flags like family history and unusual infections, as conventional lab tests are inadequate for definitive diagnosis.
- Whole-genome sequencing (WGS) enhances diagnostic efficiency and accuracy, but requires careful interpretation and collaboration among specialists to effectively manage PAD cases.
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