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Abstract
PULMONARY ATRESIA WITH VENTRICULAR SEPTAL DEFECT (PA-VSD) IN ADOLESCENCE; AN EXTREME FORM OF CLASSIC TETRALOGY OF FALLOT
Kushal Singh, Anshita Singh and Piyush*
ABSTRACT
Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in all age groups, constituting approximately 8% of the congenital heart disease overall. Classic TOF consists of ventricular septal defect, right ventricular outflow tract obstruction, right ventricular hypertrophy, and overriding aorta. In contrast, PA-VSD consists of atresia of right ventricular outflow tract along with remaining three features of classic tetralogy of Fallot. It is both clinically and radiologically distinct from regular TOF. It comprises 5–10% of all tetralogy complexes. Survival rates in TOF with pulmonary atresia without surgical repair reported in the literature are as low as 50% at 1 year of age and 8% at 10 years. We present a rare case of uncorrected TOF with pulmonary atresia in adolescent female and describing the contrast-enhanced CT and angiographic features of this entity.
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