Incidence of aortic root dilatation in pectus excavatum and its association with Marfan syndrome

Objective: To investigate the incidence of aortic root dilatation in pectus excavatum. Design: Retrospective medical record review and echocardiographic reanalysis. Setting: Morgan Stanley Children's Hospital of New York-Presbyterian. Participants: Surgical candidates with pectus excavatum (n=37) and age-matched controls (n=44) referred for an echocardiogram from 1994 to 2002. Interventions: Two-dimensional and color Doppler transthoracic echocardiograms. Outcome Measures: The aortic annulus and root were measured and z scores were calculated and compared. Medical records were reviewed for genetic evaluation. Results: Patients with pectus excavatum and agematched controls were reanalyzed. There was no difference in age, weight, height, or body surface area between patients and controls. There were no differences in the mean aortic annulus diameter, mean aortic annulus z score, or mean aortic root measurements. However, the aortic root z score was significantly higher in the pectus excavatum group compared with the controls: 0.9 (SD, 1.06) vs 0.0 (SD, 1.25) (P=.001). There were more patients with an aortic root z score of 2 or greater in the pectus excavatum group (9 of 37 patients) than in the control group (0 of 43 controls), with a calculated odds ratio of 29.7 (95% confidence interval, 1.10-1.59). Genetic evaluation was performed in 5 patients with a pectus excavatum and dilated aortic root; 2 of them received diagnoses of Marfan syndrome. Conclusions: Aortic root dilatation is more common in patients with pectus excavatum than in a control population. Echocardiographic screening may be useful in the identification of aortic root dilatation in patients with isolated pectus excavatum.