Regression of Pulmonary Vascular Disease After Therapy of Abernethy Malformation in Visceral Heterotaxy
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Date
2013
Journal Title
Journal ISSN
Volume Title
Publisher
PEDIATRIC CARDIOLOGY
Abstract
A 1-year-old boy who had left isomerism and corrected transposition of the great arteries (c-TGA) with moderate-sized ventricular septal defect, severe pulmonary artery hypertension (PAH), and pulmonary vascular disease with significant right-to-left shunting received a diagnosis of type 2 Abernethy malformation, which was partly responsible for disproportionate PAH in the child. The malformation was treated by plugging of the portosystemic shunt. Follow-up cardiac catheterization on sildenafil demonstrated significant left-to-right shunting (2.16:1) and a fall in pulmonary vascular resistance, making surgical correction possible. This case highlights the importance of searching for additional rare causes of PAH in patients with congenital heart diseases when the degree of pulmonary hypertension is disproportional to the defect size.
Description
Keywords
Cardiovascular System & Cardiology; Pediatrics
Citation
34 ,8;1882-1885