2009
DOI: 10.1016/j.jacc.2008.10.035
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The Dilated Aorta in Patients With Congenital Cardiac Defects

Abstract: Many patients with congenital cardiac disease are at risk for progressive aortic dilation. The mechanisms underlying aortic dilation in this patient cohort are described, and the similarities to the pathophysiologic alterations seen in Marfan syndrome are highlighted. Indications for treatment are discussed.

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Cited by 83 publications
(57 citation statements)
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“…There are common pathological features found in different structural defects, including abnormalities of transforming growth factor-β signaling. 78 Isolated aortic dilation often presents a dilemma for the physician with regard to recommendations for physical activity. Extreme dilation (diameter >99th percentile) is likely pathological 79 and can result in aortic aneurysm and dissection.…”
Section: Ventricular Dysfunctionmentioning
confidence: 99%
“…There are common pathological features found in different structural defects, including abnormalities of transforming growth factor-β signaling. 78 Isolated aortic dilation often presents a dilemma for the physician with regard to recommendations for physical activity. Extreme dilation (diameter >99th percentile) is likely pathological 79 and can result in aortic aneurysm and dissection.…”
Section: Ventricular Dysfunctionmentioning
confidence: 99%
“…Although the former has proved to be associated with pulmonary circulation, 24) the clinical significance of the latter remains unknown. Anatomical and functional properties of dilated aAO have been studied in patients with tetralogy of Fallot; [25][26][27] however, little data on these aortic properties and their clinical significance are available in Fontan patients. 28,29) Accordingly, we evaluated aortae in Fontan patients to clarify the aortic size and stiffness and hypothesized the following: 1) aortic size and stiffness of Fontan patients are different from those of control patients; 2) these abnormalities are associated with Fontan hemodynamics and traditional biomarkers for cardiovascular disease; and 3) aortic stiffness has a significant impact on exercise physiology.…”
mentioning
confidence: 99%
“…Расширение восходящего отдела аорты выявляется в 45-50% случаев БАК, из них около 8% осложняется диссекцией [7]. Также при БАК доста-точно часто формируется аортальный стеноз.…”
Section: Discussionunclassified