Highlights
- •Dilated and stiff ascending aortae are common in complex congenital heart disease.
- •Aortopathy was involved only in the ascending aorta, not the descending aorta.
- •Dilated and stiff ascending aortae were associated with exercise intolerance.
- •These aortic abnormalities also stratified morbidity without a synergistic impact.
- •Our data demonstrated its clinical importance as a cardiovascular risk factor.
Several studies have reported aortic dilation and increased stiffness of the ascending
aorta in patients after repair of congenital heart disease (CHD), which may be a predominant
cardiovascular risk. However, the clinical significance has not been described in
detail. In this retrospective study, 175 repaired patients with complex CHD achieving
biventricular circulation and age-matched 39 control subjects were reviewed (median
age: 14.9 and 15.7 years, respectively). We measured the diameters of the ascending
aorta and descending aorta from catheterization angiograms to yield Z-scores and stiffness
indexes (β) using diameter fluctuations corresponding to pulsatile pressures. Clinical
profile, peak oxygen uptake during the cardiopulmonary exercise test, and incidence
of unscheduled hospitalization during follow-up was also reviewed. Compared with controls,
patients with complex CHD, except for those with aortic coarctation, exhibited significant
dilation and increased stiffness of the aortic root and ascending aorta, but not of
the descending aorta. In this CHD population (n = 147, including 112 conotruncal anomalies),
exercise capacities correlated independently with the diameter Z-score and stiffness
index of the ascending aorta along with the history of repetitive thoracotomies, reduced
forced vital capacity, and right ventricular hypertension. During a follow-up period
(median 15.6 years), either dilation (Z-score >3.5) or increased stiffness (β >6.0)
of the ascending aorta stratified morbidity, but no synergistic impact was detected.
In conclusion, in repaired patients with complex CHD, a stiffened and dilated ascending
aorta was frequently found, exerting significant adverse impacts on diminished exercise
capacity and morbidity.
Graphical Abstract
Graphical Abstract
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Article info
Publication history
Published online: May 26, 2020
Received in revised form:
May 14,
2020
Received:
January 19,
2020
Footnotes
Grant: This work was partially supported by Takeda Science Foundation.
Identification
Copyright
© 2020 Elsevier Inc. All rights reserved.
