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Updated: Jul 19, 2026

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Pulmonary atresia with intact ventricular septum associated with congenitally corrected transposition
C S Knight1, S H Litovsky, F B Pearce
1Division of Anatomic Pathology, Department of Pathology, University of Alabama at Birmingham, North Pavilion 3547, 1802 6th Avenue South, Birmingham, AL 34249, USA.
Abstract:
We present a rare case of pulmonary atresia with intact septum associated with congenitally corrected transposition. The patient was a nondysmorphic female infant. Despite a right Blalock-Taussig shunt on day 13 of life and a balloon atrial septostomy on day 23, she remained ventilator dependent with poor systemic ventricular function. The patient died at the age of 160 days, and postmortem findings are presented. The literature on this condition is reviewed, and the possibility that the left-sided morphologic right ventricle could not sustain the systemic circulation is discussed.
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