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Management and outcome of Ebstein's anomaly in children
- Angela Oxenius, Christine H. Attenhofer Jost, René Prêtre, Hitendu Dave, Urs Bauersfeld, Oliver Kretschmar, Burkhardt Seifert, Christian Balmer, Emanuela R. Valsangiacomo Buechel
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- Journal:
- Cardiology in the Young / Volume 23 / Issue 1 / February 2013
- Published online by Cambridge University Press:
- 15 March 2012, pp. 27-34
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Objectives
To 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.
Remodelling after surgical repair of atrial septal defects within the oval fossa
- Christine H. Attenhofer Jost, Erwin Oechslin, Burkhardt Seifert, Friedrich Maly, Renata Fatio, Juraj Turina, Rolf Jenni
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- Journal:
- Cardiology in the Young / Volume 12 / Issue 6 / December 2002
- Published online by Cambridge University Press:
- 15 August 2006, pp. 506-512
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In a retrospective study, we analysed the data from 101 adults with echocardiographic follow-up after surgical repair of defects within the oval fossa at a mean age of 35 ± 17 years; 56% of the cohort being above the age of 30 years. Mean age at follow-up was 44 ± 18 years, and length of follow-up was up to 40 years (11 ± 12 years). At follow-up, atrial fibrillation or flutter was present in one quarter. Dilation of the right atrium, found in 64%, of the left atrium, found in 44%, and of the right ventricle, found in 29%, were also frequent, as well as pulmonary arterial hypertension, which was found in 30%. Diminished right ventricular ejection fraction, in contrast, was very rare, found only in 1%, and abnormal left ventricular ejection fraction was not encountered. By multivariate analysis, predictors for right or left atrial, or right ventricular, dilation were age at follow-up, degree of tricuspid regurgitation, pulmonary hypertension, and/or atrial fibrillation. In a subset of 21 patients in sinus rhythm, we correlated prospectively the diastolic and systolic function of both ventricles with levels of brain natriuretic peptide, comparing values to those of 20 age-matched controls with a mean age of 46 ± 14 years. Levels of brain natriuretic peptide were significantly higher in patients than in controls (p = 0.006), and correlated significantly with diastolic dysfunction (p = 0.007) and left atrial size (p < 0.0001). In the long-term follow-up after surgical repair of defect within the oval fossa, therefore, complete normalization of heart size and function is rare. Despite preserved systolic function, persistent diastolic dysfunction is common and is associated with elevated levels of brain natriuretic peptide, which may explain the late occurrence of atrial arrhythmias.