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Dilation of the ascending aorta in childhood: 4 cases without obvious predisposing disease

Published online by Cambridge University Press:  15 August 2006

Jean-Pierre Pfammatter
Affiliation:
Pediatric Cardiology and Cardiovascular Surgery, University Hospital Berne, Switzerland
Mladen Pavlovic
Affiliation:
Pediatric Cardiology and Cardiovascular Surgery, University Hospital Berne, Switzerland
Pascal Berdat
Affiliation:
Pediatric Cardiology and Cardiovascular Surgery, University Hospital Berne, Switzerland
Thierry Carrel
Affiliation:
Pediatric Cardiology and Cardiovascular Surgery, University Hospital Berne, Switzerland

Abstract

Dilation of the ascending aorta is rare in childhood. When seen, it is usually associated with some form of connective tissue disease or predisposing cardiac malformations, especially an aortic valve with two leaflets. We describe four children in whom significant dilation of the ascending aorta was encountered as an incidental finding. No patient had any sign of an associated connective tissue disease, nor did we detect any predisposing cardiac anomalies. One patient had undergone surgical ligation of the arterial duct in infancy, whilst another had undergone repair of aortic coarctation, also in infancy. A third child has had repair of an atrioventricular septal defect with exclusively atrial shunting, whereas the fourth patient had a structurally normal heart. The aortic valve had three leaflets, and was functionally normal in all. The dilation of the ascending aorta was progressive in all patients, and finally surgical treatment was recommended, relying on the guidelines established for the management of patients affected with the Marfan syndrome.

Type
Original Article
Copyright
2001 Cambridge University Press

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