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Korean Circulation Journal Feb 2024
PubMed: 38346696
DOI: 10.4070/kcj.2024.0007 -
Heart, Lung & Circulation Mar 2024
Topics: Humans; Tricuspid Valve; Ebstein Anomaly; Mitral Valve Prolapse; Tricuspid Valve Insufficiency; Echocardiography
PubMed: 38341315
DOI: 10.1016/j.hlc.2023.12.021 -
World Journal For Pediatric &... Mar 2024
Topics: Humans; Ebstein Anomaly
PubMed: 38334052
DOI: 10.1177/21501351241227895 -
Journal of Interventional Cardiac... Jun 2024Ebstein's anomaly of the tricuspid valve (EA) is an uncommon congenital cardiac malformation. It can present with atrioventricular tachycardia (AVRT), atrioventricular... (Review)
Review
Ebstein's anomaly of the tricuspid valve (EA) is an uncommon congenital cardiac malformation. It can present with atrioventricular tachycardia (AVRT), atrioventricular nodal re-entrant tachycardia (AVNRT), atrial arrhythmias, and rarely with ventricular tachycardia. The 12-lead electrocardiogram (ECG) is critically important and often diagnostic even prior to an electrophysiology study (EPS). Due to its complex anatomy, it poses particular challenges for mapping and ablation, even for an experienced electrophysiologist. In this review, we aim to provide insight into the electrophysiological perspective of EA and an in-depth analysis of the various arrhythmias encountered in diverse clinical scenarios.
Topics: Ebstein Anomaly; Humans; Electrocardiography; Electrophysiologic Techniques, Cardiac; Catheter Ablation; Female; Male; Tachycardia, Atrioventricular Nodal Reentry
PubMed: 38289561
DOI: 10.1007/s10840-024-01744-8 -
HeartRhythm Case Reports Jan 2024
PubMed: 38264116
DOI: 10.1016/j.hrcr.2023.10.023 -
World Journal For Pediatric &... Mar 2024Ebstein anomaly is a rare congenital anomaly of the tricuspid valve which presents challenges to cardiac surgeons due to the spectrum of the disease and the technical...
BACKGROUND
Ebstein anomaly is a rare congenital anomaly of the tricuspid valve which presents challenges to cardiac surgeons due to the spectrum of the disease and the technical difficulty of valve repair. The natural history of the anomaly differs between patients presenting in the neonatal period to those presenting in adulthood.
METHODS
A retrospective review of all patients >15 years of age with Ebstein anomaly, undergoing surgery on the tricuspid valve at 6 centers across Australia and New Zealand was performed. Patients from 1985 to 2019 were included in the study.
RESULTS
A total of 125 patients were included in the study, 76 patients (60%) undergoing tricuspid valve repair, of which 23 patients underwent a Cone repair and 49 (40%) had a tricuspid valve replacement. The mean follow-up was 7.9 ± 7.3 years. Postoperatively, early mortality was 3 patients (2%) and 10-year survival was 91.5%. A postoperative pacemaker was required in 24 patients (19%). Reoperation was required in 21 patients (17%). There was no statistically significant difference in survival or reoperation between patients who underwent repair or replacement of the tricuspid valve; however, with a small number of patients in long-term follow-up.
CONCLUSION
Older children and adult patients undergoing surgery for Ebstein anomaly in Australia and New Zealand experience good medium-term postoperative survival. Repair of the valve is achieved in a significant proportion of patients with increasing use and success with the Cone repair technique.
Topics: Child; Infant, Newborn; Adult; Humans; Adolescent; Ebstein Anomaly; New Zealand; Treatment Outcome; Tricuspid Valve; Australia
PubMed: 38263637
DOI: 10.1177/21501351231189279 -
Cardiology in the Young Apr 2024Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe...
Primary liver tumours in neonates with single-ventricle palliation are exceedingly rare. We present the first reported case of neonatal hepatoblastoma with severe Ebstein's anomaly following Starnes procedure. The patient's postoperative course highlights the challenges and complications in simultaneous management of these diagnoses. Transition from shunted single-ventricle physiology to bidirectional cavopulmonary connection improved end-organ function, permitting more aggressive hepatic malignancy treatment.
Topics: Infant, Newborn; Humans; Ebstein Anomaly; Hepatoblastoma; Univentricular Heart; Liver Neoplasms
PubMed: 38247377
DOI: 10.1017/S1047951124000039 -
Journal of Neonatal-perinatal Medicine 2024A circular shunt is a poor prognostic factor associated with Ebstein's anomaly. Targeting the constriction of the ductus arteriosus (DA) in order to limit or resolve the...
A circular shunt is a poor prognostic factor associated with Ebstein's anomaly. Targeting the constriction of the ductus arteriosus (DA) in order to limit or resolve the circular shunt, has been shown to improve fetal outcomes. Prenatal non-steroidal anti-inflammatory drugs (NSAIDs) have been known to constrict the DA. Recently, prenatal NSAIDs have been used for that purpose in the treatment of circular shunt. Limited research shows that it may be an effective treatment leading to improved fetal outcomes. In this article, we did an extensive review of literature to describe this therapy's effectiveness and outcomes. 82% of fetuses were able to achieve ductal constriction with prenatal NSAID therapy. For fetuses who achieved ductal constriction, fetal demise was less likely (6%) when compared to those who were unable to achieve the same (50%). Of all the fetuses with hydrops, 50% had resoluation of hydrops with prenatal NSAID treatment.
Topics: Pregnancy; Female; Humans; Ebstein Anomaly; Anti-Inflammatory Agents, Non-Steroidal; Ductus Arteriosus, Patent; Ductus Arteriosus; Edema
PubMed: 38217614
DOI: 10.3233/NPM-230040