2 results
Does superior caval vein pressure impact head growth in Fontan circulation?
- Tina Trachsel, Christian Balmer, Håkan Wåhlander, Roland Weber, Hitendu Dave, Andrea Poretti, Oliver Kretschmar, Anna Cavigelli-Brunner
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- Journal:
- Cardiology in the Young / Volume 26 / Issue 7 / October 2016
- Published online by Cambridge University Press:
- 15 January 2016, pp. 1327-1332
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Background
Patients with bidirectional cavopulmonary anastomosis have unphysiologically high superior caval vein pressure as it equals pulmonary artery pressure. Elevated superior caval vein pressure may cause communicating hydrocephalus and macrocephaly. This study analysed whether there exists an association between head circumference and superior caval vein pressure in patients with single ventricle physiology.
MethodsWe carried out a retrospective analysis of infants undergoing Fontan completion at our institution from 2007 to 2013. Superior caval vein pressures were measured during routine catheterisation before bidirectional cavopulmonary anastomosis and Fontan completion as well as head circumference, adjusted to longitudinal age-dependent percentiles.
ResultsWe included 74 infants in our study. Median ages at bidirectional cavopulmonary anastomosis and Fontan were 4.8 (1.6–12) and 27.9 (7–40.6) months, respectively. Head circumference showed significant growth from bidirectional cavopulmonary anastomosis until Fontan completion (7th (0–100th) versus 20th (0–100th) percentile). There was no correlation between superior caval vein pressure and head circumference before Fontan (R2=0.001). Children with lower differences in superior caval vein pressures between pre-bidirectional cavopulmonary anastomosis and pre-Fontan catheterisations showed increased growth of head circumference (R2=0.19).
ConclusionsPatients with moderately elevated superior caval vein pressure associated with single ventricle physiology did not have a tendency to develop macrocephaly. There is no correlation between superior caval vein pressure before Fontan and head circumference, but between bidirectional cavopulmonary anastomosis and Fontan head circumference increases significantly. This may be explained by catch-up growth of head circumference in patients with more favourable haemodynamics and concomitant venous pressures in the lower range. Further studies with focus on high superior caval vein pressures are needed to exclude or prove a correlation.
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.