Transplantation proceedings · 2021
Resolution of Precapillary Pulmonary Hypertension After Liver Transplantation for Hereditary Hemorrhagic Telangiectasia: Systematic Review and Case Report.
Клинический случайСистематический обзорСтатьяНазвание и аннотация: Перевод в очереди
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Perrodin Stéphanie F, Vogt Andreas P, Berzigotti Annalisa, Kim-Fuchs Corina, Luedi Markus M, Candinas Daniel, Banz Vanessa M
Аннотация
BACKGROUND: Pulmonary hypertension (PH) is a known complication of hereditary hemorrhagic telangiectasia (HHT) in patients with hepatic arteriovenous malformations (HAVM). Orthotopic liver transplantation (OLT) is a recognized treatment of HAVM in HHT, but its effect on PH has not been reported in detail before.
METHODS: Systematic review on HHT patients with pre- or postcapillary PH who underwent OLT and report of a case.
RESULTS: Twenty-one patients were included from 7 articles, all case reports or case series. All had high-output cardiac failure prior to OLT. Two patients had precapillary PH, both related to ALK1 mutations. All patients but 1 showed significant improvement or complete resolution of PH after transplantation. One patient died of acute cardiac failure postoperatively. We also report the case of a 72-year-old woman with type 3 HHT and severe mixed pre- and postcapillary PH. The patient presented with multiple HAVM, left-to-right shunting, and severe but partially reversible combined pre- and postcapillary PH, without ALK1 mutation. After recurrent cholangitis episodes, liver abscesses, and severe obstruction of the right-sided biliary tree, an interdisciplinary decision was taken to proceed with OLT despite PH. Intraoperatively, PH resolved almost instantly after hepatic artery ligation and hepatectomy.
CONCLUSIONS: In our patient, OLT completely abrogated mixed pre- and postcapillary PH. Based on this systematic review, we suggest that OLT should be considered a viable treatment option in patients with HHT, HAVM, and mixed pre- and postcapillary PH, featuring cardiac failure and drug responsive PH, rather than being seen as a major risk factor for cardiopulmonary complications.
METHODS: Systematic review on HHT patients with pre- or postcapillary PH who underwent OLT and report of a case.
RESULTS: Twenty-one patients were included from 7 articles, all case reports or case series. All had high-output cardiac failure prior to OLT. Two patients had precapillary PH, both related to ALK1 mutations. All patients but 1 showed significant improvement or complete resolution of PH after transplantation. One patient died of acute cardiac failure postoperatively. We also report the case of a 72-year-old woman with type 3 HHT and severe mixed pre- and postcapillary PH. The patient presented with multiple HAVM, left-to-right shunting, and severe but partially reversible combined pre- and postcapillary PH, without ALK1 mutation. After recurrent cholangitis episodes, liver abscesses, and severe obstruction of the right-sided biliary tree, an interdisciplinary decision was taken to proceed with OLT despite PH. Intraoperatively, PH resolved almost instantly after hepatic artery ligation and hepatectomy.
CONCLUSIONS: In our patient, OLT completely abrogated mixed pre- and postcapillary PH. Based on this systematic review, we suggest that OLT should be considered a viable treatment option in patients with HHT, HAVM, and mixed pre- and postcapillary PH, featuring cardiac failure and drug responsive PH, rather than being seen as a major risk factor for cardiopulmonary complications.