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

doi:10.1016/j.jtcvs.2008.12.027

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

Daniëlle Robbers-Visser, MD^a^,b, Livia Kapusta, MD, PhD^g, Lennie van Osch-Gevers, MD, PhD^a, Jan L.M. Strengers, MD, PhD^h, Eric Boersma, PhD^c, Yolanda B. de Rijke, PhD^d, Frans Boomsma, PhD^e, Ad J.J.C. Bogers, MD, PhD^f, Willem A. Helbing, MD, PhD^a^,b^,_*

^a Department of Pediatrics, Division of Cardiology, Erasmus MC—Sophia Children's Hospital, Rotterdam, The Netherlands
^b Department of Radiology, Erasmus MC, Rotterdam, The Netherlands
^c Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands
^d Department of Clinical Chemistry, Erasmus MC, Rotterdam, The Netherlands
^e Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands
^f Department of Cardiothoracic Surgery, Erasmus MC, Rotterdam, The Netherlands
^g Children's Heart Center, UMC St Radboud, Nijmegen, The Netherlands
^h Department of Pediatric Cardiology, University MC Utrecht—Wilhelmina Children's Hospital, Utrecht, The Netherlands

Received for publication July 15, 2008; revisions received December 11, 2008; accepted for publication December 25, 2008.

^_* Address for reprints: W. A. Helbing, MD, PhD, Erasmus MC–Sophia Children's Hospital, Department of Paediatric Cardiology, Sp-2429, PO Box 2060, 3000 CB Rotterdam, The Netherlands. (Email: w.a.helbing{at}erasmusmc.nl).

Objective: This study assessed clinical condition at midterm follow-upafter total cavopulmonary connection for a functionally univentricularheart performed on children younger than 5 years.

Methods: Thirty-four Fontan patients (median age 10.4 years, range 6.8–20.7years, 22 boys, median follow-up 7.8 years, 5.0–17.8 years)underwent electrocardiography, Holter monitoring, bicycle exercisetesting, cardiac magnetic resonance imaging, and N-terminalprohormone brain natriuretic peptide (NT-pro-BNP) analysis.

Results: Twenty-three patients (68%) were in sinus rhythm. Holter monitoringdemonstrated 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 maximaloxygen uptake (69% of normal) were all significantly lower inthe Fontan group than in a control group (P < .001). Variablesof submaximal exercise indicated less efficient oxygen uptakeduring exercise in all Fontan patients. Ejection fraction waslower than in control subjects (59% ± 13% vs 69% ±5%, P < .001). Mean end-diastolic and end-systolic volumesand ventricular mass were higher than in control subjects (P< .001). Mean NT-pro-BNP levels were increased relative toreference values, but only 8 patients had levels above the upperreference limit.

Conclusion: At midterm follow-up, Fontan patients were in acceptable clinicalcondition, with preserved global ventricular function, moderatelydecreased exercise capacity, and NT-pro-BNP levels within referencerange. Systemic ventricular mass was elevated, however, suggestingcontractility–afterload mismatch. Long-term consequencesfor ventricular function merit further investigation.

Abbreviations and Acronyms BNP = brain natriuretic peptide;EDVI = end-diastolic volume index; EF = ejection fraction; ESVI= end-systolic volume index; LV = left ventricle; MRI = magneticresonance imaging; NT-pro-BNP = N-terminal prohormone brainnatriuretic peptide; OUES = oxygen uptake efficiency slope;RQ = respiratory quotient; RV = right ventricle; TCPC = totalcavopulmonary connection;

= pulmonary ventilation;

= oxygen consumption

Congenital Heart Disease

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