Ebstein’s anomaly with ‘reversible’ functional pulmonary atresia

We present the case of an infant with prenatal diagnosis, at 32 weeks gestation, of Ebstein’s anomaly without anterograde flow from right ventricular to pulmonary atresia (PA)—functional PA with flow reversal in the ductus arteriosus. Prostaglandin E1 was started after birth. Chest X-ray showed severe cardiomegaly and echocardiogram confirmed Ebstein’s anomaly with a thickened non-opening pulmonary valve without anterograde flow but with mild regurgitation. Multidisciplinary team decision was to progressively reduce prostaglandins and have an expectant attitude. Peripheral oxygen saturation above 85% was maintained and serial echocardiograms documented progressive reduction of the ductus arteriosus and the opening of the pulmonic valve cusps, with the development of anterograde flow. The newborn was discharged at day 19 of life without the need for any intervention, and at last follow-up remains asymptomatic, with anterograde normal flow in the pulmonary valve.

Download Full-text

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

Cardiology in the Young ◽

10.1017/s1047951120003820 ◽

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.

Download Full-text

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

Cardiology in the Young ◽

10.1017/s1047951121000081 ◽

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.

Download Full-text

Long-term follow-up after repair of Ebstein's anomaly

European Journal of Cardio-Thoracic Surgery ◽

10.1016/j.ejcts.2008.03.048 ◽

2008 ◽

Vol 34 (1) ◽

pp. 48-54 ◽

Cited By ~ 15

Author(s):

Meindert Palmen ◽

Peter L. de Jong ◽

Loes M.A. Klieverik ◽

Angelique C. Venema ◽

Folkert J. Meijboom ◽

...

Keyword(s):

Ebstein’S Anomaly ◽

Ebstein's Anomaly ◽

Long Term Follow Up

Download Full-text

Mid-term Outcome Of Surgical Treatment In Pediatric Patients With Ebstein's Anomaly: A Single-center Cohort Study.

10.21203/rs.3.rs-42297/v1 ◽

2020 ◽

Author(s):

Jia-chen Li ◽

Xian-chao Jiang ◽

Si-meng Zhang ◽

Jin-yang Liu ◽

Ya-juan Zhang ◽

...

Keyword(s):

Surgical Treatment ◽

Reoperation Rate ◽

Term Effect ◽

Ebstein’S Anomaly ◽

Biventricular Repair ◽

Ebstein's Anomaly ◽

Long Term Follow Up ◽

Surgical Treatments

Abstract Background: Ebstein’s anomaly is a malformation of the tricuspid valve and myopathy of the right ventricle. Surgery is now the main treatment for the defect. To summarize our surgical results and experience based on patients with Ebstein’s anomaly who were under 7 years of age and treated with different surgical treatments.Materials and Methods: From January 2010 to December 2019, 80 patients under 7 years old who were diagnosed of Ebstein’s anomaly and underwent different surgical treatments were consecutively enrolled and followed up in detail. Results: The median age of the 80 patients at the time of surgery was 3.63 years. Sixty-four (80.00%) patients underwent biventricular repair while 13 (16.25%) underwent 1.5-ventricle repair. With the median follow-up 27.50 months, the long-term survival of the total cohort, 1.5-ventricular repair and biventricular repair was 82.35%, 91.67% and 100%, respectively. The long-term freedom from reoperation rate was 97.50%, 92.31% and 98.44%, respectively. Mild, moderate and severe TR before surgery occurred in 6 (7.50%), 18 (22.50%) and 56 (70.00%), respectively. The early outcomes of 78 patients were 65 (83.33%), 11 (14.11%) and 2 (2.56%); the mid-term outcomes of 72 patients were 49 (68.06%), 19 (26.38%) and 4 (5.56%). Both early and long-term valve regurgitation were significantly decreased (p< 0.001) compared with preoperative condition. No more severe regurgitation occurred (p=0.404), though some early mild regurgitation became acceptable moderate regurgitation during long-term follow-up (p=0.036). Compared with Carpentier procedure, cone procedure had better long-term effect, while the effect of whole-valve technique needed more operation and long-term follow-up.Conclusion: The reoperation rate and mid-term mortality of surgical treatment for Ebstein’s anomaly were both low, tricuspid regurgitation was significantly improved during mid-term follow up. Cone procedure had the best mid-term effect among anatomic repair.

Download Full-text

Pulmonary atresia with intact septum: the use of Conquest Pro coronary guidewire for perforation of atretic valve and subsequent interventions

Cardiology in the Young ◽

10.1017/s1047951112000595 ◽

2012 ◽

Vol 23 (2) ◽

pp. 197-202 ◽

Cited By ~ 8

Author(s):

Mazeni Alwi ◽

Rahmat R. Budi ◽

Marhisham Che Mood ◽

Ming C. Leong ◽

Hasri Samion

Keyword(s):

Patent Ductus Arteriosus ◽

Balloon Dilatation ◽

Pulmonary Valve ◽

Ductus Arteriosus ◽

Pulmonary Atresia ◽

Right Ventricular Outflow Tract ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Total Occlusion ◽

Patent Ductus

AbstractObjectiveTo determine the feasibility and safety of the Conquest Pro wire as an alternative to radiofrequency wire for perforation of atretic pulmonary valve and subsequent balloon dilatation and patent ductus arteriosus stenting in patients with pulmonary atresia with intact ventricular septum.BackgroundRadiofrequency valvotomy and balloon dilatation has become the standard of care for pulmonary atresia with intact ventricular septum in many institutions today.MethodsWe report eight consecutive patients in whom we used the Conquest Pro coronary guidewire, a stiff wire normally reserved for revascularisation of coronary lesions with chronic total occlusion, for perforation of atretic pulmonary valve and subsequent balloon dilatation, and stenting of the patent ductus arteriosus.ResultsPerforation of atretic pulmonary valve was successful in seven out of eight cases. Radiofrequency valvotomy was employed after failure of perforation by the Conquest Pro wire in one case where the right ventricular outflow tract was broad based and tapered towards the pulmonary valve, and was heavily trabeculated. Failure of the Conquest Pro wire to perforate the pulmonary valve plate was mainly attributed by the failure to engage the wire at the correct position.ConclusionThe Conquest Pro wire for perforation and subsequent interventions in the more straightforward cases of pulmonary atresia with intact ventricular septum is effective and safe, simplifying the entire procedure. However, the radiofrequency generator and wires remain essential tools in the paediatric interventional catheter laboratory.

Download Full-text

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

Cardiology in the Young ◽

10.1017/s1047951114001747 ◽

2014 ◽

Vol 25 (6) ◽

pp. 1119-1123 ◽

Cited By ~ 9

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.

Download Full-text