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J Thorac Cardiovasc Surg 2007;133:470-477
© 2007 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Clinical condition at mid-to-late follow-up after transatrial–transpulmonary repair of tetralogy of Fallot

Jochem van den Berg, MDa,b, Wim C. Hop, PhDc, Jan L.M. Strengers, MD, PhDd, Johan C. de Jongste, MD, PhDe, Lennie van Osch-Gevers, MD, PhDa, Folkert J. Meijboom, MD, PhDf, Peter M.T. Pattynama, MD, PhDb, Ad J.J.C. Bogers, MD, PhDg, Willem A. Helbing, MD, PhDa,b,*

a Department of Paediatric Cardiology, Erasmus MC, Rotterdam, The Netherlands
b Department of Radiology, Erasmus MC, Rotterdam, The Netherlands
c Department of Epidemiology and Biostatistics, Erasmus MC, Rotterdam, The Netherlands
e Department of Paediatric Pulmonology, Erasmus MC, Rotterdam, The Netherlands
f Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands
g Department of Cardiothoracic Surgery, Erasmus MC, Rotterdam, The Netherlands
d Department of Paediatric Cardiology, University MC, Wilhelmina Children’s Hospital, Utrecht, The Netherlands

Received for publication July 3, 2006; revisions received September 28, 2006; accepted for publication October 9, 2006.

* Address for reprints: W.A. Helbing, MD, PhD, Department of Paediatric Cardiology, Erasmus MC—Sophia Children’s Hospital, Sp-2429, Dr Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands (Email: w.a.helbing{at}erasmusmc.nl).

OBJECTIVES: To assess the clinical condition at mid-to-late follow-up in tetralogy of Fallot corrected by a transatrial–transpulmonary approach at a young age and to identify risk factors associated with right ventricular dilation/dysfunction and with decreased exercise tolerance.

METHODS: Patients with tetralogy of Fallot underwent cardiac magnetic resonance imaging, maximal bicycle ergometry, electrocardiography, Holter monitoring, and spirometry. Multivariate linear regression analyses were used to determine independent predictors for selected clinical parameters.

RESULTS: Fifty-nine patients (mean ± SD), age at repair 0.9 ± 0.5 years, interval since repair 14 ± 5 years, were included. The median pulmonary regurgitant fraction was 32% (0%-57%). Compared with published data on healthy controls, Fallot patients had significantly larger right ventricular end-diastolic and end-systolic volumes and smaller right ventricular and left ventricular ejection fractions. Maximum oxygen consumption was 97% ± 17% and maximum workload 89% ± 13% of predicted. Median QRS duration was 110 ms (82–161 ms). No important ventricular arrhythmias were found. Compared with patients without a transannular patch, patients with a patch had more pulmonary regurgitation, a larger right ventricle, worse right ventricular and left ventricular ejection fractions, but comparable exercise capacity. Multivariate regression analysis identified the following independent determinants for larger right ventricular volumes: longer interval since repair, longer QRS duration, and higher pulmonary regurgitation percentage. The following were independent determinants for smaller right ventricular ejection fraction: abnormal right ventricular outflow tract wall motion, longer interval since repair, and longer QRS duration. For smaller maximum oxygen consumption, the independent determinants were smaller right ventricular ejection fraction and longer QRS duration.

CONCLUSIONS: At mid-to-late follow-up, clinical condition in tetralogy of Fallot corrected according to contemporary surgical approaches appears well preserved. However, even these patients show right ventricular dilation and dysfunction associated with impaired functional capacity. Abnormalities relate to right ventricular outflow tract motion abnormalities, longer interval since repair, longer QRS duration, and more severe pulmonary regurgitation.

Abbreviations and Acronyms CMR = cardiac magnetic resonance imaging; FVC = forced vital capacity; LV = left ventricular; PR = pulmonary regurgitation; RQ = respiratory quotient; RV = right ventricular; TAP = transannular patch; TE = echo time; TOF = tetralogy of Fallot; VO 2max = maximum oxygen consumption; VSD = ventricular septal defect






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