Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort

2008 ◽  
Vol 168 (6) ◽  
pp. 685-690 ◽  
Author(s):  
Ya-Mei Chang ◽  
Jou-Kou Wang ◽  
Sheunn-Nan Chiu ◽  
Ming-Tai Lin ◽  
En-Ting Wu ◽  
...  
Keyword(s):  
Clinical Spectrum ◽  
Ebstein’S Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ebstein's Anomaly ◽  
Asian Cohort

Clinical Spectrum and Long-term Outcome of 183 Cases of Ebstein's Anomaly, Experience of Mexican Population

2020 ◽  
Vol 51 (4) ◽  
pp. 336-342
Author(s):  
Héctor Herrera-Bello ◽  
Nydia Ávila-Vanzzini ◽  
Juan Francisco Fritche-Salazar ◽  
Jorge Kuri-Alfaro ◽  
Manuel B.A. Gaxiola-Macias ◽  
...  
Keyword(s):  
Mexican Population ◽  
Clinical Spectrum ◽  
Ebstein’S Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ebstein's Anomaly

Abstract 20041: Single Ventricle Outcome Following Neonatal Palliation of Severe Ebstein’s Anomaly With Modified Starnes Procedure

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
S. Ram Kumar ◽  
Nathan Noh ◽  
Novell Castillo ◽  
Brian Fagan ◽  
Grace Kung ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Nyha Class ◽  
Pacemaker Implantation ◽  
Outcome Data ◽  
Left Ventricular ◽  
Ebstein’S Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ebstein's Anomaly

Background: We have previously shown that neonates in profound cardiogenic shock due to severe Ebstein’s anomaly can be successfully salvaged with fenestrated right ventricular (RV) exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single ventricle management in these patients is not known. Methods: We retrospectively reviewed the records of 26 patients who underwent neonatal Starnes procedure between 1989 and 2011. Patient demographics, clinical variables and outcome data were collected. Data is presented as mean ± standard errors or median (interquartile ranges). Results: 26 patients (12, 46% boys) underwent Starnes procedure at 7 (5-9) days of life. All were intubated and on prostacyclin infusion, 24 (92%) were inotrope-dependent and 23 (88%) had no antegrade flow from the RV. Two patients had had prior intervention (one tricuspid annuloplasty and one shunt alone). Three patients underwent non-fenestrated RV exclusion, two (67%) of whom died. Of the remaining 23, 3 (13%) died during the same hospitalization. The 21 neonatal survivors have been followed for 7 (6-8) years. One patient died after Glenn. The remaining 20 have successfully undergone Fontan completion with an indexed pulmonary resistance of 1.8 (1.2-2.3) W/m2 and mean pulmonary pressure of 12 (9-18) mm Hg. At last follow-up, all patients have normal left ventricular function, and all but one patient are in NYHA Class I symptoms. Two patients have required pacemaker implantation, while the rest are in sinus rhythm. Survival at 1, 5 and 10 years are 81±4%, 77±3% and 77±3%, respectively. Conclusion: Long-term single ventricle outcomes amongst neonatal survivors of modified Starnes procedure are excellent. There is reliable remodeling of the excluded RV and excellent function of the left ventricle.

scholarly journals Management and outcome of Ebstein's anomaly in children

2012 ◽  
Vol 23 (1) ◽  
pp. 27-34 ◽  
Author(s):  
Angela Oxenius ◽  
Christine H. Attenhofer Jost ◽  
René Prêtre ◽  
Hitendu Dave ◽  
Urs Bauersfeld ◽  
...  
Keyword(s):  
Cardiac Surgery ◽  
Operative Mortality ◽  
Ebstein’S Anomaly ◽  
Tertiary Institution ◽  
Term Outcome ◽  
Term Survival ◽  
Long Term Outcome ◽  
Ebstein's Anomaly ◽  
Included Patient

AbstractObjectivesTo assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly.BackgroundData on long-term outcome of children with Ebstein's anomaly are scarce.MethodsRetrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention.ResultsA total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1–16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%.ConclusionIn children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.

Early timing of surgical intervention in patients with Ebstein's anomaly predicts superior long-term outcome

2009 ◽  
Vol 56 (S 01) ◽  
Author(s):  
CC Badiu ◽  
C Schreiber ◽  
J Hörer ◽  
DJ Ruzicka ◽  
M Wottke ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Ebstein’S Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ebstein's Anomaly ◽  
Early Timing

scholarly journals Effect of Ebstein's anomaly on short- and long-term outcome of surgically treated patients with Wolff-Parkinson-White syndrome.

Circulation ◽  
1992 ◽  
Vol 86 (4) ◽  
pp. 1147-1155 ◽  
Author(s):  
J C Pressley ◽  
J M Wharton ◽  
A S Tang ◽  
J E Lowe ◽  
J J Gallagher ◽  
...  
Keyword(s):  
Ebstein’S Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
White Syndrome ◽  
Ebstein's Anomaly ◽  
Short And Long Term ◽  
Wolff Parkinson White Syndrome

scholarly journals Early timing of surgical intervention in patients with Ebstein's anomaly predicts superior long-term outcome☆

2010 ◽  
Vol 37 (1) ◽  
pp. 186-192 ◽  
Author(s):  
Catalin Constantin Badiu ◽  
Christian Schreiber ◽  
Jürgen Hörer ◽  
Daniel Junichiro Ruzicka ◽  
Michael Wottke ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Ebstein’S Anomaly ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Ebstein's Anomaly ◽  
Early Timing

Long-term Outcome and Clinical Spectrum of 73 Pediatric Patients With Mitochondrial Diseases

PEDIATRICS ◽  
2007 ◽  
Vol 119 (4) ◽  
pp. 722-733 ◽  
Author(s):  
F.-G. Debray ◽  
M. Lambert ◽  
I. Chevalier ◽  
Y. Robitaille ◽  
J.-C. Decarie ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Mitochondrial Diseases ◽  
Clinical Spectrum ◽  
Term Outcome ◽  
Long Term Outcome
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