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J Thorac Cardiovasc Surg 2007;133:1094-1096
© 2007 The American Association for Thoracic Surgery

Brief Communication

The negative impact of Alagille syndrome on survival of infants with pulmonary atresia

Gillian M. Blue, MScia,b, Jean M. Mah, MBBSc, Andrew D. Cole, BAppScia, Vanita Lal, PhDa, Meredith J. Wilson, MBBS, FRACP, MBioethb, Richard B. Chard, BDS, MBBS, FRACSa,c, Gary F. Sholler, MBBS, FRACPa,c, Richard E. Hawker, MBBS, FRACPa, Megan C. Sherwood, MBBS, FRACPa,c, David S. Winlaw, MD, FRACSa,c,*

a Kids Heart Research and Adolph Basser Cardiac Institute, The Children’s Hospital at Westmead, Sydney, Australia
b Department of Clinical Genetics, The Children’s Hospital at Westmead, Sydney, Australia
c Discipline of Paediatrics and Child Health, Faculty of Medicine, University of Sydney, The Children’s Hospital at Westmead, Sydney, Australia.

Received for publication December 12, 2006; accepted for publication December 18, 2006.

* Address for reprints: Dr David Winlaw, Paediatric Cardiac Surgeon, Head, Kids Heart Research, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead NSW 2145, Australia. (Email: davidw@chw.edu.au).

The first 20% of the full text of this article appears below.

Alagille syndrome (AGS) is a complex disorder with multisystemic involvement, including the liver, heart, kidneys, cerebral vasculature, skeleton, eyes, and face.1Go A structural heart defect is one of the diagnostic criteria for AGS. These vary in severity, with peripheral pulmonary arterial stenosis being a common problem. Pulmonary atresia (PA) is a rare presentation in AGS, but we were impressed by the poor outcome of such infants following review of our institutional experience over the last 20 years. The information has particular relevance in this era where prenatal diagnosis of both AGS and PA can be made.

Clinical Summary

A number of information sources including cardiac, genetic, and gastroenterology departmental databases and hospital medical records were searched and cross-referenced to identify patients with AGS and/or PA between 1985 and 2004. We identified 26 patients with AGS and 505 patients with PA, 5 of whom had both diagnoses. . . . [Full Text of this Article]




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[Abstract] [Full Text] [PDF]


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