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An obstructive bicuspid aortic valve in the setting of tetralogy of Fallot with pulmonary atresia: a rare combination

Published online by Cambridge University Press:  20 January 2005

Cengiz Ermis ,
Monesha Gupta-Malhotra  and
Jack L. Titus
Cengiz Ermis
Affiliation:
Division of Cardiology, University of Minnesota, Minneapolis, MN, USA
Monesha Gupta-Malhotra
Affiliation:
Division of Pediatric Cardiology, University of Minnesota, Minneapolis, MN, USA
Jack L. Titus
Affiliation:
Department of Pathology, Jesse E. Edwards Registry of Cardiovascular Disease, United Hospital, St Paul, MN, USA
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Abstract

As far as we are aware, a bicuspid aortic valve has not previously been reported in the setting of tetralogy of Fallot and pulmonary atresia. We describe this association in a newborn who presented with a murmur and cyanosis. Echocardiography showed tetralogy of Fallot with pulmonary atresia, and also a moderately stenotic bicuspid aortic valve. The patient underwent open-heart surgery guided by transesophageal echocardiography. Postoperatively, there was only mild obstruction across both outflow tracts. We have also reviewed the pertinent data from our Jesse E. Edwards Registry of Cardiovascular Disease to establish the incidence of bicuspid aortic valve in the setting of obstruction of the right ventricular outflow tract, finding the association in no patients with pulmonary atresia and tetralogy of Fallot, in 0.7% of those with tetralogy of Fallot and pulmonary stenosis, but in 6.6% of those with pulmonary atresia and intact ventricular septum.

Keywords

Pulmonary atresia with ventricular septal defectpulmonary atresia with intact ventricular septumcongenital cardiac malformations
Type
Brief Report
Information
Cardiology in the Young , Volume 14 , Issue 1 , February 2004 , pp. 99 - 101
Copyright
© 2004 Cambridge University Press

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