Document Detail

Clinical outcome 5 to 18 years after the Fontan operation performed on children younger than 5 years.
MedLine Citation:
PMID:  19577062     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: This study assessed clinical condition at midterm follow-up after total cavopulmonary connection for a functionally univentricular heart performed on children younger than 5 years. METHODS: Thirty-four Fontan patients (median age 10.4 years, range 6.8-20.7 years, 22 boys, median follow-up 7.8 years, 5.0-17.8 years) underwent electrocardiography, Holter monitoring, bicycle exercise testing, cardiac magnetic resonance imaging, and N-terminal prohormone brain natriuretic peptide (NT-pro-BNP) analysis. RESULTS: Twenty-three patients (68%) were in sinus rhythm. Holter monitoring demonstrated normal mean heart rate, low maximal heart rate, and no clinically significant arrhythmias or sinus node dysfunction. With maximal bicycle ergometry (n = 19), maximum workload (60% of normal), maximum heart rate (90% of normal), and maximal oxygen uptake (69% of normal) were all significantly lower in the Fontan group than in a control group (P < .001). Variables of submaximal exercise indicated less efficient oxygen uptake during exercise in all Fontan patients. Ejection fraction was lower than in control subjects (59% +/- 13% vs 69% +/- 5%, P < .001). Mean end-diastolic and end-systolic volumes and ventricular mass were higher than in control subjects (P < .001). Mean NT-pro-BNP levels were increased relative to reference values, but only 8 patients had levels above the upper reference limit. CONCLUSION: At midterm follow-up, Fontan patients were in acceptable clinical condition, with preserved global ventricular function, moderately decreased exercise capacity, and NT-pro-BNP levels within reference range. Systemic ventricular mass was elevated, however, suggesting contractility-afterload mismatch. Long-term consequences for ventricular function merit further investigation.
Daniëlle Robbers-Visser; Livia Kapusta; Lennie van Osch-Gevers; Jan L M Strengers; Eric Boersma; Yolanda B de Rijke; Frans Boomsma; Ad J J C Bogers; Willem A Helbing
Publication Detail:
Type:  Journal Article     Date:  2009-02-23
Journal Detail:
Title:  The Journal of thoracic and cardiovascular surgery     Volume:  138     ISSN:  1097-685X     ISO Abbreviation:  J. Thorac. Cardiovasc. Surg.     Publication Date:  2009 Jul 
Date Detail:
Created Date:  2009-07-06     Completed Date:  2009-07-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376343     Medline TA:  J Thorac Cardiovasc Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  89-95     Citation Subset:  AIM; IM    
Department of Pediatrics, Division of Cardiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
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MeSH Terms
Exercise Test
Follow-Up Studies
Fontan Procedure*
Heart Defects, Congenital / physiopathology,  surgery*
Heart Rate
Magnetic Resonance Imaging
Myocardium / pathology
Natriuretic Peptide, Brain / blood
Oxygen Consumption
Peptide Fragments / blood
Young Adult
Reg. No./Substance:
0/Peptide Fragments; 0/pro-brain natriuretic peptide (1-76); 114471-18-0/Natriuretic Peptide, Brain

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