Surgical management of symptomatic neonates with Ebstein’s anomaly: choice of operation

2014 ◽  
Vol 25 (6) ◽  
pp. 1119-1123 ◽  
Author(s):  
Christopher J. Knott-Craig ◽  
Thittamaranahalli Kariyappa S. Kumar ◽  
Alejandro R. Arevalo ◽  
Vijaya M. Joshi
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Surgical Management ◽  
Single Ventricle ◽  
Pulmonary Atresia ◽  
Early Mortality ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Single Ventricle Palliation ◽  
Artery Conduit

AbstractObjective:Symptomatic neonates with Ebstein’s anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset.Methods:A total of 32 consecutive symptomatic neonates with Ebstein’s anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients’ weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein’s anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes’ palliation and 2 Blalock–Taussig shunts. Six recent patients with Ebstein’s anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair.Results:Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein’s anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4).Conclusions:Surgical management of neonates with Ebstein’s anomaly remains challenging. For neonates with Ebstein’s anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.

Point ablation for macro-reentrant ventricular tachycardia associated with Ebstein’s anomaly and pulmonary atresia: a case report

2020 ◽  
pp. 1-3
Author(s):  
Keiko Toyohara ◽  
Yasuko Tomizawa ◽  
Morio Shoda
Keyword(s):  
Ventricular Tachycardia ◽  
Case Report ◽  
Catheter Ablation ◽  
Right Ventricle ◽  
Low Voltage ◽  
Pulmonary Atresia ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Slow Conduction ◽  
Hypoplastic Right Ventricle

Abstract We report a case with Ebstein’s anomaly and pulmonary atresia with sustained monomorphic ventricular tachycardia in a patient without a ventriculotomy history. In the low voltage area between the atrialised right ventricle and hypoplastic right ventricle, there was a ventricular tachycardia substrate and slow conduction. The tachycardia circuit was eliminated by a point catheter ablation at the area with diastolic fractionated potentials.

scholarly journals Critical Ebstein’s anomaly with circular shunt: from successful fetal therapy with non-steroidal anti-inflammatory drugs to biventricular repair using Da Silva cone technique

2021 ◽  
pp. 1-4
Author(s):  
Lilian M. Lopes ◽  
Rodrigo F. Bezerra ◽  
Jose Pedro da Silva ◽  
Luciana da Fonseca da Silva
Keyword(s):  
Single Ventricle ◽  
Ductus Arteriosus ◽  
Ebstein’S Anomaly ◽  
Biventricular Repair ◽  
Fetal Demise ◽  
Ebstein's Anomaly ◽  
Gestational Week ◽  
Inflammatory Drugs ◽  
Anti Inflammatory ◽  
Single Ventricle Palliation

Abstract We report an innovative treatment strategy for fetal Ebstein’s anomaly with a circular shunt. We used transplacental non-steroidal anti-inflammatory drugs, at the 29th gestational week, to constrict the ductus arteriosus avoiding fetal demise. We addressed the critical neonate with an urgent Starnes procedure. Finally, instead of following the usual single-ventricle palliation pathway after the Starnes procedure, we achieved successful two-ventricle repair with the cone technique at 5 month old.

Aortic or Pulmonary Valved Homograft Right Ventricle to Pulmonary Artery Conduit in the Norwood Procedure

2019 ◽  
Vol 10 (4) ◽  
pp. 499-501
Author(s):  
Teimour Nasirov ◽  
Katsuhide Maeda ◽  
Olaf Reinhartz
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Outcome Data ◽  
Percutaneous Intervention ◽  
Early Mortality ◽  
The Other ◽  
Stage I ◽  
Norwood Procedure ◽  
Norwood Procedures ◽  
Artery Conduit

Background: Several modifications of the Norwood procedure utilizing valved right ventricle to pulmonary artery conduits have recently been reported. Our group has been using aortic or pulmonary valved homografts combined with PTFE tube grafts for now 16 years. Methods: In this report, we review our technique in detail and describe any changes that have occurred over the years. We provide detailed illustrations of our preferred surgical technique, report outcome data, and compare it to the other conduit options available. Results: Between 2006 and 2015, 130 stage I Norwood procedures were performed at our institution, 100 of them using valved conduits. Our technique is described and illustrated in detail. Early mortality was 15%. Postoperative percutaneous intervention on the conduit was required in 29% of cases. Conclusions: While a randomized trial comparing different valved conduits is lacking, we believe a composite conduit made from homograft aortic or pulmonary valves and PTFE tube grafts is an excellent choice in stage I Norwood procedure.

Modified Starnes procedure as a bridge to 2-ventricle repair in neonatal Ebstein’s anomaly: A paradigm shift

2021 ◽  
Keyword(s):  
Tricuspid Valve ◽  
Single Ventricle ◽  
Hemodynamic Instability ◽  
Ebstein’S Anomaly ◽  
Tricuspid Valve Repair ◽  
Valve Regurgitation ◽  
Valve Repair ◽  
Tricuspid Valve Regurgitation ◽  
Ebstein's Anomaly ◽  
Single Ventricle Palliation

Severe tricuspid valve regurgitation secondary to Ebstein’s anomaly represents several challenges in neonates. It can result in significant respiratory and/or hemodynamic compromise that mandates urgent interventions. When conservative management fails, 2 surgical options are available: tricuspid valve repair or single ventricle palliation. The overall results of neonatal tricuspid valve repair are unsatisfactory especially in sick neonates and those with preoperative hemodynamic instability. Single ventricle palliation utilizing the Starnes procedure with right ventricular exclusion provides a quicker way to improve hemodynamics and allows rapid decompression of the right ventricle but carries the long-term disadvantages of the single ventricle pathway. We were recently faced with a challenging case of neonatal Ebstein’s anomaly resulting in severe tricuspid valve regurgitation (TR) and significant hemodynamic and respiratory instability. We performed an initial stage I palliation with a modified Starnes’ procedure, which allowed stabilization and rapid recovery of the patient to be followed 5 months later with conversion to 2-ventricle repair using the cone technique. We believe combining these 2 strategies for suitable neonatal candidates may be a useful technique that should be considered in the algorithm for neonatal Ebstein’s anomaly.

Creation of an intraatrial tunnel to produce a total cavo-pulmonary connection in a patient with dominant right ventricle, totally anomalous pulmonary venous connection, and pulmonary atresia with non-confluent pulmonary arteries

2008 ◽  
Vol 18 (6) ◽  
pp. 641-643
Author(s):  
Koichi Sughimoto ◽  
Mitsuru Aoki ◽  
Tadashi Fujiwara
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Single Ventricle ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Surgical Strategy ◽  
Anomalous Pulmonary Venous Connection ◽  
Goretex Patch

AbstractThe surgical strategy for patients having a functionally single ventricle associated with totally anomalous pulmonary venous connection and pulmonary atresia with non-confluent pulmonary artery has yet to be agreed. We created an intraatrial tunnel to produce a total cavo-pulmonary connection in such a patient, also creating a confluence for the pulmonary arteries. By minimizing the use of the GoreTex patch, the patient was able to discontinue the use of warfarin.

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