Clinical outcome 5 to 18 years after the Fontan operation performed on children younger than 5 years.
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.


CONCLUSIONS
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.
Robbers-Visser
Daniëlle
D
Department of Pediatrics, Division of Cardiology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
Kapusta
Livia
L
van Osch-Gevers
Lennie
L
Strengers
Jan L M
JL
Boersma
Eric
E
de Rijke
Yolanda B
YB
Boomsma
Frans
F
Bogers
Ad J J C
AJ
Helbing
Willem A
WA
D000293:Adolescent; D000328:Adult; D002648:Child; D004562:Electrocardiography; D005080:Exercise Test; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000601:surgery*; D006339:Heart Rate; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009206:Myocardium / Q000473:pathology; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010101:Oxygen Consumption; D010446:Peptide Fragments / Q000097:blood; D055815:Young Adult
D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain