Regression of Pulmonary Vascular Disease After Therapy of Abernethy Malformation in Visceral Heterotaxy
Tóm tắt
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.
Tài liệu tham khảo
Alvarez AE, Ribeiro AF, Hessel G et al (2002) Abernethy malformation: one of the etiologies of hepatopulmonary syndrome. Pediatr Pulmonol 34:391–394
Franchi-Abella S, Branchereau S, Lambert V et al (2010) Complications of congenital portosystemic shunts in children: therapeutic options and outcomes. J Pediatr Gastroenterol Nutr 51:322–333
Herve P, Lebrec D, Brenot F et al (1998) Pulmonary vascular diseases in portal hypertension. Eur Respir J 11:1153–1166
Hori T, Yonekawa Y, Okamoto S et al (2011) Pediatric orthotopic living-donor liver transplantation cures pulmonary hypertension caused by Abernethy malformation type Ib. Pediatr Transplant 15:e47–e52
Hu G-H, Shen L-G, Yang J, Mei J-H, Zhu Y-F (2008) Insight into congenital absence of the portal vein: is it rare? World J Gastroenterol 14:5969–5979
Imamura H, Momose T, Kitabayashi H et al (2000) Pulmonary hypertension as a result of asymptomatic portosystemic shunt. Jpn Circ J 64:471–473
Morgan G, Superina R (1994) Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies. J Pediatr Surg 29:1239–1241
Ohno T, Munuechi J, Ihara K et al (2008) Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics 121:e892–e899 Epub 24 March 2008