Adolescents with complex congenital heart disease (CHD) who have undergone surgical repair were frequently found to have a stiffened and dilated ascending aorta, which may affect exercise capacity and increase morbidity, according to a study published in the American Journal of Cardiology.

This was a single-center retrospective review of 175 patients with complex CHD who achieved biventricular circulation after surgical repair (median age, 14.9 years). All patients in this cohort underwent diagnostic cardiac catheterization >1 year after surgical repair and were clinically stable without inotropic agents. A total of 39 age-matched control individuals (median age, 15.7 years) had a history of Kawasaki disease without stenotic coronary arterial lesions.

Measurements of the diameters of the ascending aorta and descending aorta were conducted on catheterization angiograms to produce stiffness indexes. The patients’ clinical profiles, peak oxygen uptake during a cardiopulmonary exercise test, and the incidence of unscheduled hospitalization during follow up were reviewed. A total of 137 patients (78%) underwent pulmonary function testing with conventional spirometry within 1 month of the cardiopulmonary exercise test. The occurrence of adverse clinical events, a composite of all-cause death, during follow up was monitored.

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During a median follow-up period of 15.6 years (interquartile range, 11.8–17.5 years), 6% (n=9) of patients died, and 37% (n=55) patients experienced clinical events. Some of the events recorded included additional cardiac surgeries for pulmonary stenosis or insufficiency (n=22), arrhythmia and pacemaker implantation (n=16), faintness or malaise (n=6), right-sided heart failure (n=3), prosthetic aortic valve replacement (n=2), angina suggestive of myocardial ischemia (n=2), hemorrhagic event (n=2), and noncardiac surgery (n=2).

Dilation and increased stiffness of the aortic root and ascending aorta was found to be frequent in patients with complex CHD compared with control individuals. No dilation or stiffness was detected in the descending aorta. Patients with stiff and/or dilated ascending aorta had significant morbidity compared with patients with a mild abnormality in the ascending aorta (P =.018). Exercise capacity was significantly reduced in patients with stiff and/or dilated ascending aorta compared with patients with a mild abnormality in this region (P <.001).

Limitations of this study include its retrospective and single-center design, and the fact that the control population had increased left ventricular end-diastolic pressure.

“Among this complex CHD population, dilated and stiff [ascending aorta] predicted reduced exercise capacity and morbidity, suggesting the clinical importance of the aortic abnormality as a cardiovascular risk factor,” noted the study authors.


Hayama Y, Ohuchi H, Negishi J, et al. Effect of stiffened and dilated ascending aorta on aerobic exercise capacity in repaired patients with complex congenital heart disease [published online May 26, 2020]. Am J Cardiol. doi:10.1016/j.amjcard.2020.05.028