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Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: Pulmonary hypertension and pulmonary vascular dilatation
Author(s) -
Law Y. M.,
Mack C. L.,
Sokol R. J.,
Rice M.,
Parsley L.,
Ivy D.
Publication year - 2011
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2010.01355.x
Subject(s) - medicine , pulmonary hypertension , liver transplantation , portal hypertension , cardiology , heterotaxy , surgery , vascular disease , transplantation , heart disease , cirrhosis
Law YM, Mack CL, Sokol RJ, Rice M, Parsley L, Ivy D. Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: Pulmonary hypertension and pulmonary vascular dilatation.
Pediatr Transplantation 2011: 15: E162–E168. © 2010 John Wiley & Sons A/S. Abstract: 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.