scholarly journals Recent Surgical Outcomes of Ebstein’s Anomaly: A Focus on Changes in Outcomes Due to Cone Repair

2020 ◽  
Author(s):  
Eun-Young Choi ◽  
Eun Sun Kim ◽  
Jung Yoon Kim ◽  
Seong-Ho Kim ◽  
Jae Hong Lim ◽  
...  
Keyword(s):  
Atrioventricular Block ◽  
Tricuspid Valve ◽  
Ebstein’S Anomaly ◽  
Valve Regurgitation ◽  
Complete Atrioventricular Block ◽  
Tricuspid Valve Regurgitation ◽  
Ebstein's Anomaly ◽  
Cone Repair ◽  
Repair Group ◽  
Complete Atrioventricular

Abstract Background: Ebstein’s anomaly exhibits a wide variety of clinical features, and therefore, proposing a standardized treatment for it is difficult. This study was conducted to determine whether Cone repair, which has been implemented in our hospital since 2008, is more effective than conventional repair.Methods: We retrospectively analyzed the clinical information of patients with Ebstein’s anomaly who were followed-up at the hospital from 2000 to 2019. A total of 61 patients who had undergone tricuspid valve repair after 2000 were divided into the conventional and Cone repair groups and their clinical outcomes were compared.Results: Of the 170 patients, 82 (48.2%) patients received surgical treatment for the tricuspid valve, whereas 75 patients received only medical treatment. The median follow-up duration was 5.89 years. After surgery, tricuspid valve regurgitation decreased and aortic stroke volume increased in both the Cone and conventional repair groups. In the Cone repair group, no mortality and postoperative complete atrioventricular block occurred and significantly fewer cases of moderate to severe tricuspid valve regurgitation were noted after surgery compared with the conventional repair group.Conclusions: Cone repair is thought to be a method with less mortality and less occurrence of complete atrioventricular block than conventional repair.

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.

The impact of tricuspid valve regurgitation severity on exercise capacity and cardiac-related hospitalisations among adults with non-operated Ebstein’s anomaly

2019 ◽  
Vol 29 (06) ◽  
pp. 800-807
Author(s):  
Jonathan Buber ◽  
Ori Vatury ◽  
Robert Klempfner ◽  
Shai Tejman-Yarden
Keyword(s):  
Oxygen Consumption ◽  
Exercise Capacity ◽  
Tricuspid Valve ◽  
Tricuspid Regurgitation ◽  
Ebstein’S Anomaly ◽  
Valve Regurgitation ◽  
Tricuspid Valve Regurgitation ◽  
Ebstein's Anomaly ◽  
Cardiopulmonary Exercise ◽  
Lesion Severity

AbstractBackground:Tricuspid valve regurgitation is an inherent part of Ebstein’s anomaly, yet whether the severity of the regurgitation further impairs exercise capacity and contributes to long-term morbidity on top of the lesion severity per se is unknown.Methods:To evaluate for this potential effect, we included 30 patients with Ebstein’s anomaly who did not undergo any form of surgical interventions and had a cardiopulmonary exercise test and echocardiographic studies in this retrospective analysis. Echocardiographic studies and cardiopulmonary exercise tests were critically reviewed for lesion severity grade, tricuspid regurgitation degree, and exercise parameters. Cardiac-related hospitalisations were recorded from computerised medical records and during clinic visits.Results:Fourteen patients (47%) had moderate and 8 (27%) had severe regurgitation. Patients with ≥ moderate regurgitation exhibited significantly lower exercise capacity (median % predicted maximal oxygen consumption, 62 versus 79%, p = 0.03) and venilatory efficiency at exercise. When stratifying exercise results by regurgitation degree, a stepwise decrease in oxygen consumption and ventilatory efficiency with increasing regurgitation severity was observed, regardless of the anatomic lesion severity. During a median follow-up of 4.6 years, > moderate tricuspid regurgitation was associated with significantly lower cumulative probability of freedom from cardiac hospitalisations.Conclusions:We report that among non-operated Ebstein’s anomaly patients, greater tricuspid regurgitation severity was associated with worse exercise capacity and with overall higher probability of cardiac-related hospitalisations independent from the underlying lesion severity.

Cone repair for Ebstein’s anomaly and atrial fibrillation ablation in an adult patient

2021 ◽  
Keyword(s):  
Atrial Fibrillation ◽  
Tricuspid Valve ◽  
Pulmonary Veins ◽  
Maze Procedure ◽  
Right Atrial ◽  
Ebstein’S Anomaly ◽  
Ebstein's Anomaly ◽  
Previously Treated ◽  
Cone Repair ◽  
The Right

We present a 52-year-old woman with Ebstein’s anomaly not previously treated. In this subset of patients, there are no clear guidelines regarding the best surgical strategy for treating the tricuspid valve: replace it or repair it. In this case, extensive repair of the tricuspid valve and the right ventricle is achieved using the cone repair technique popularized by Dr. José Pedro Da Silva. Because the patient also presented with symptomatic paroxysmal atrial fibrillation, a right atrial maze procedure combined with isolation of the pulmonary veins was performed using both radiofrequency and cryotherapy. At the last follow-up, 2 years after the repair, the patient is asymptomatic and maintains sinus rhythm. The last echocardiogram showed mild tricuspid regurgitation with normal right ventricular function.

Emergency trans-oesophageal ventricular pacing in a child

2004 ◽  
Vol 14 (3) ◽  
pp. 333-334 ◽  
Author(s):  
Elisabeth Villain ◽  
Younes Boudjemline ◽  
Damien Bonnet
Keyword(s):  
Atrioventricular Block ◽  
Tricuspid Valve ◽  
Coronary Sinus ◽  
Ventricular Pacing ◽  
Complete Atrioventricular Block ◽  
Complete Atrioventricular

We report our experience with an 8-year-old boy with complete atrioventricular block and syncopal bradycardia who required urgent pacing. Each attempt to cross the tricuspid valve with a femoral lead triggered ventricular standstill, followed by fibrillation, and pacing through the coronary sinus failed. Successful ventricular pacing was finally achieved through the oesophagus, allowing subsequent implantation of a transvenous pacemaker.

scholarly journals Surgical management for an adult, female patient, with Ebstein Anomaly on Tricuspid Valve that has Subvalvular membrane with severe PS and multiple VSDs-A case report.

2020 ◽  
Vol 23 (6) ◽  
pp. E781-E785
Author(s):  
Saikat Das Gupta ◽  
Mauin Uddin ◽  
Siddhartha Shankar Howlader ◽  
Prodip Kumar Biswas ◽  
Mohammed Kabiruzzaman ◽  
...  
Keyword(s):  
Tricuspid Valve ◽  
Septal Defect ◽  
Surgical Intervention ◽  
Pulmonary Stenosis ◽  
Exercise Intolerance ◽  
Ebstein’S Anomaly ◽  
Ebstein Anomaly ◽  
Tricuspid Valve Regurgitation ◽  
Ebstein's Anomaly ◽  
Adult Female Patient

Ebstein's anomaly is a rare and complexed heart defect that affects the tricuspid valve and is accountable for around 1% of congenital cardiac abnormalities. It is one of the most common congenital causes of tricuspid valve regurgitation. Ebstein's anomaly is often diagnosed prenatally due to its severe cardiomegaly. Some individuals with this anomaly do not experience any complications until adulthood and even then its mostly minor complaints like exercise intolerance.  Atrial septal defect is most commonly (70-90%) associated with Ebstein's anomaly. However, ventricular septal defect (VSD) can be associated with 2-6% of the cases. This particular report presents a case of surgical intervention for a 20 years old female with Ebstein's anomaly that had multiple VSD's and a severe Pulmonary Stenosis (PS).

Sign in / Sign up

Export Citation Format

Share Document