• Heynric B Grotenhuis, Frederic Dallaire, Inez M Verpalen, Michelle J E van den Akker, Luc Mertens, and Mark K Friedberg.
• Division of Cardiology, Department of Pediatrics, Wilhelmina Children's Hospital, University of Utrecht, The Netherlands (H.B.G.).
• Circ Cardiovasc Imaging. 2018 Dec 1; 11 (12): e007611.

BackgroundThe study objective was to assess the severity, progression rate, and risk factors for aortic root dilatation (ARD) in pediatric tetralogy of Fallot (TOF) and to study its relationship to complications including aortic regurgitation.Methods And ResultsSeven hundred sixty-eight TOF patients were studied with echocardiography (median age at repair 6.1 months; interquartile range, 4.5-11.3 months) and compared with 304 controls. Five hundred ninety-two (77%) had usual TOF, 41 (5.3%) TOF-type double outlet right ventricle, and 135 (17.6%) TOF with pulmonary atresia. Median follow-up was 3.7 years (interquartile range, 0.5-6.9 years, total follow-up 3002 patients-years). Aortic root dimensions were enlarged at TOF diagnosis and during follow-up. Mean aortic valve annulus Z score at first postoperative echo was 3.3±2.7, associated with TOF type (TOF1 Z score unit per year) was observed in <1% of patients. Of the 768 patients, only 3 (0.39%) developed moderate/severe aortic regurgitation and 2 required aortic surgery for subvalvar aortic stenosis and aortic dissection, respectively.ConclusionsARD is common before and after TOF repair, but our data suggest that aortic root dimensions remain stable in the majority of pediatric TOF patients. Progressive ARD and aortic-related complications are distinctly uncommon during childhood.

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