Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: pulmonary hypertension and pulmonary vascular dilatation. Pediatr Transplant 2011 Dec;15(8):E162-8
Date
06/23/2010Pubmed ID
20565694Pubmed Central ID
PMC3203339DOI
10.1111/j.1399-3046.2010.01355.xScopus ID
2-s2.0-82155197271 (requires institutional sign-in at Scopus site) 21 CitationsAbstract
HPS and PPHTN are unusual and challenging pulmonary manifestations of liver disease. We report two pediatric cases in association with heterotaxy polysplenia syndrome and congenital absence of the portal vein. Both patients were symptomatic and hemodynamically compromised and required aggressive medical therapy. One patient with PPHTN alone achieved a successful liver transplant. The second child presented with combined HPS and PPHTN and exhibited a different evolution of pulmonary vascular disease. These cases illustrate associations that must be entertained in the setting of heterotaxy syndrome, cyanosis, or pulmonary hypertension and how strategic medical combined with surgical management can provide a good outcome.
Author List
Law YM, Mack CL, Sokol RJ, Rice M, Parsley L, Ivy DAuthor
Cara Lynn Mack MD Chief, Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Abnormalities, MultipleChild, Preschool
Dilatation, Pathologic
Female
Hepatopulmonary Syndrome
Heterotaxy Syndrome
Humans
Hypertension, Pulmonary
Infant
Liver Transplantation
Male
Portal Vein
Pulmonary Artery
