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

• Published in: Cardiology in the young Vol. 14; no. 1; pp. 99 - 101
• Main Authors: Ermis, Cengiz, Gupta-Malhotra, Monesha, Titus, Jack L.
• Format: Journal Article
• Language: English
• Published: Cambridge, UK Cambridge University Press 01.02.2004
• Subjects: Angiography; Aortic Valve - abnormalities; Aortic Valve - surgery; Brief Report; Cardiology; Cardiovascular disease; congenital cardiac malformations; Echocardiography; Female; Heart; Heart Defects, Congenital - complications; Heart Defects, Congenital - diagnosis; Heart Defects, Congenital - surgery; Humans; Infant, Newborn; Mitral Valve - abnormalities; Mitral Valve - surgery; Pulmonary arteries; Pulmonary Atresia - complications; pulmonary atresia with intact ventricular septum; Pulmonary atresia with ventricular septal defect; Tetralogy of Fallot - complications; Veins & arteries; congenital cardiac malformations; pulmonary atresia with intact ventricular septum; Pulmonary atresia with ventricular septal defect
• ISSN: 1047-9511; 1467-1107
• DOI: 10.1017/S1047951104001209

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.