Aortic root dilatation after repair of tetralogy of Fallot: pathology from the past?

Abstract

Repair of tetralogy of Fallot (TOF) has been performed since 19541 and adult survival is expected.2 Long-term problems include arrhythmias, both supraventricular and ventricular, and residual hemodynamic problems, which are generally well tolerated for many years; these include residual ventricular septal defect and pulmonary stenosis but, most commonly, pulmonary regurgitation, with its consequent right ventricular dysfunction and tricuspid regurgitation. Although the most common reason for repeat surgery in the adult after TOF repair relates to problems in the right ventricular outflow tract, the aortic root is often forgotten. In this issue of Circulation , Niwa et al3 report a series of 32 patients who demonstrated a dilated aortic root after surgical repair of tetralogy of Fallot (TOF). Aortic root size measured echocardiographically was >1.5 expected diameter by standard nomogram. This “dilator” group comprised almost 15% of their TOF patient population. Characteristics of these patients with TOF compared with age-matched controls included a higher prevalence of pulmonary atresia, a longer shunt-to-repair interval, and a right aortic arch. Other features were consequent on the dilated aorta rather than causal, moderate-to-severe aortic regurgitation and a larger left ventricle both echocardiographically and radiographically. Two of these patients needed aortic valve replacement and root repair. See p 1374 These observations are not new. Progressive aortic root dilatation after complete uncomplicated repair of TOF has been noted previously,4 and others have reported aortic regurgitation.5–7⇓⇓ In the majority …