title: Cardiopulmonary manifestations of portovenous shunts from congenital absence of the portal vein: pulmonary hypertension and pulmonary vascular dilatation.
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.
authors.affiliation: Department of Pediatrics, Seattle Children's Hospital, Seattle, Washington 98105, USA. yuk.law@seattlechildrens.org
authors.forename: Y M
authors.initials: YM
authors.lastname: Law
authors.forename: C L
authors.initials: CL
authors.lastname: Mack
authors.forename: R J
authors.initials: RJ
authors.lastname: Sokol
authors.forename: M
authors.initials: M
authors.lastname: Rice
authors.forename: L
authors.initials: L
authors.lastname: Parsley
authors.forename: D
authors.initials: D
authors.lastname: Ivy
mesh_terms: D000015:Abnormalities, Multiple; D002675:Child, Preschool; D004108:Dilatation, Pathologic; D005260:Female; D020065:Hepatopulmonary Syndrome / Q000150:complications* / Q000503:physiopathology; D059446:Heterotaxy Syndrome / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000503:physiopathology / Q000628:therapy; D007223:Infant; D016031:Liver Transplantation; D008297:Male; D011169:Portal Vein / Q000002:abnormalities*; D011651:Pulmonary Artery / Q000473:pathology*