Published Online
on August 19, 2002

A more recent version of this article appeared on September 10, 2002

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Submitted on April 26, 2002
Revised on June 17, 2002
Accepted on June 19, 2002

Progressive Aortic Root Dilatation in Adults Late After Repair of Tetralogy of Fallot

Koichiro Niwa MD^*, Samuel C. Siu MD, SM, Gary D. Webb MD, and Michael A. Gatzoulis MD, PhD

From Toronto Congenital Cardiac Center for Adults, Toronto, Ontario, Canada. Dr Niwa is now at the Adult Congenital Heart Program, Department of Pediatrics, Chiba Cardiovascular Center, Chiba, Japan. Dr Gatzoulis is now at the Adult Congenital Heart Program, Department of Cardiology, Royal Brompton Hospital, London, UK.

^* To whom correspondence should be addressed. E-mail: KNIWA{at}aol.com.

Background—Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its dynamic nature in adults with repaired TOF.

Methods and Results—Of 216 patients with TOF repair who underwent echocardiography in 1997, we identified 32 patients (mean age, 36±8.0 years) with AoRo dilatation, defined as ratio of observed to expected AoRo size by standard nomogram >1.5 (group A), and 54 TOF controls, matched for age with AoRo ratio <1.5 (group B), who underwent at least 1 previous echocardiogram in the preceding 10 years. Mean indexed AoRo size (cm/m²) in 1997 was 2.5±0.5 in group A and 1.7±0.2 in group B (P<0.0001). AoRo rate of change (mm/year) from the first to 1997 study (mean interval, 5.2±3.8 years) was 1.7±3.8 in group A and 0.03±1.6 in group B (P=0.001). Patients from group A had a longer shunt-to-repair interval (P=0.048) with a higher prevalence of pulmonary atresia (P<0.0001), right aortic arch (P=0.03), moderate to severe AR (P=0.002), aortic valve replacement (P=0.02), larger cardiothoracic ratio (P=0.02), and increased left ventricular end-diastolic dimensions (P=0.002).

Conclusions—A subset of adult TOF exhibits ongoing dilatation of AoRo late after repair. This dilatation relates to previous long-standing volume overload of AoRo and possibly to intrinsic properties of AoRo and may lead to AR. Meticulous follow-up of AoRo after TOF repair is recommended.

Key words: tetralogy of Fallot • aneurysm • aorta • follow-up studies • heart defects, congenital

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