Detailed three-dimensional cardiac segmentations using cardiac computed tomography (CT) data is technically feasible in patients with Ebstein anomaly, but its complementary role has not been evaluated. This single-center, retrospective study was aimed to evaluate the complementary role of cardiac CT ventricular volumetry in evaluating the severity of Ebstein anomaly. Preoperative cardiac CT ventricular volumetry was performed in 21 children with Ebstein anomaly. CT-based ventricular functional measures were compared between Carpentier types, and between definitive surgical repair types. The Celermajer severity index measured with echocardiography was correlated with CT-based functional parameters. Total right ventricle (RV) and functional RV (fRV) volumes, fRV fraction, fRV/left ventricle (LV) volume ratio, and end-diastolic CT severity index demonstrated statistically significant differences between Carpentier type A/B and Carpentier type C/D (p < 0.05). The Celermajer severity index measured with echocardiography showed a high positive correlation with the end-diastolic CT severity index (R = 0.720, p < 0.002). There were no statistically significant differences in both echocardiography- and CT-based functional measures between patients with biventricular repair and patients with one-and-a-half or univentricular repair (p > 0.05). Compared with echocardiography, cardiac CT ventricular volumetry can provide the severity of Ebstein anomaly objectively and may be used in select patients when echocardiographic results are inconclusive or inconsistent.
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http://dx.doi.org/10.1007/s00246-023-03342-3 | DOI Listing |
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.
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 PDFJ Cardiothorac Vasc Anesth
December 2024
Department of Cardiothoracic Surgery, National Heart Center, Royal Hospital, Muscat, Oman.
A circular shunt, initially described by Shone et al. in 1962, refers to abnormal blood recirculation through complete intracardiac or intra- and extracardiac communications, bypassing the capillary beds. This pathophysiological condition is most commonly associated with complex congenital heart defects, such as Ebstein's malformation, pulmonary atresia, Gerbode defect, and so on.
View Article and Find Full Text PDFAm J Med Genet A
December 2024
Division of Medical Genetics, Kanagawa Children's Medical Center, Yokohama, Japan.
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.
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