Liver Transplantation as Curative Treatment in Severe Pulmonary Hypertension due to Hepatic Vascular Malformations in Rendu-Osler-Weber Disease

INTRODUCTIONHereditary haemorragic telangiectasia (HHT) is an autosomal dominant disease characterised by development of arterio-venous malformations (AVM) more common in mucosa, brain, lung, liver and gastrointestinal tract. Pulmonary hypertension (PH) is defined as mean pulmonary arterial pressure...

Full description

Saved in:

Bibliographic Details
Published in:	Transplantation Vol. 102 Suppl 7S-1; no. Supplement 7; p. S890
Main Authors:	Caballero, Javier Martínez, Pulido, Jorge J Calvo, Marcacuzco Quinto, Alberto Alejandro AA, González, Lucia L González, Alonso, Iago I Justo, Maestro, Oscar O Caso, Del Pozo, Pilar P, Padilla, Isabel I Montilla, Molero, Felix F Cambra, Municio, Alejandro A Manrique, Sesma, Alvaro A García, Romero, Luis Carlos C Jiménez
Format:	Journal Article
Language:	English
Published:	Copyright Wolters Kluwer Health, Inc. All rights reserved 01-07-2018
Online Access:	Get full text
Tags:	Add Tag No Tags, Be the first to tag this record!

Description
Summary:	INTRODUCTIONHereditary haemorragic telangiectasia (HHT) is an autosomal dominant disease characterised by development of arterio-venous malformations (AVM) more common in mucosa, brain, lung, liver and gastrointestinal tract. Pulmonary hypertension (PH) is defined as mean pulmonary arterial pressure (mPAP) > 25 mmHg. Liver transplatation is still controversial in this patients. MATERIALSMETHODSWoman, 38 years old, with personal history of eclampsia, HELLP síndrome and HHT type 2 (Rendu-Osler-Weber) with exon 9 in the ALK1 gen mutation that presents as systemic manifestations self-limited epistaxis and liver AVM with mainly arterio-systemic intrahepatic shunt which lead to high output cardiac arrest and moderate to severe PH with a NYHA II functional class.Lung and brain AVM were not observed in radiologic tests and bubble test was positive due to extracardiac shunt (>6 heart-beats). RESULTSDue to the high risk related to liver transplantation (LT) in this patient, transarterial embolization of liver vascular malformations was performed first, with a 24% reducción in cardiac output (table 1) and development of several complications as intranodal reentry tachycardia and ischemic hepatitis. Because of partial improvement treatment with bevacizumab was iniciated, despite this, the patient suffered recurrent episodes of cardiac arrest and liver function deterioration (MELD 25).In July 2016, orthotopic LT was performed with a cold ischemia time of 355 min and a warm ischemia time of 40 min, during which he bled profusely requiring massive blood transfusion.After LT, a 53.2% reduction in the cardiac output was observed in a right catheterism, because extracardiac shunt was eliminated. Furthermore, a pulmonary vascular resistance (PVR) reduction, wich means an absence of vascular branch remodelation, and a normalization in mPAP was shown; which demonstrate the resolution of HP after LT (table 2).(Table is included in full-text article.)(Table is included in full-text article.) CONCLUSION- LT might be a curative treatment in patients with haemorrhagic vascular diseases if severe HP and cardiac arrest are due to liver AVM- Liver AVM embolization in moderate-severe HP with elevated cardiac output has mild results- Diagnosis of Lung AVM causing HP, by angio-TC or angiography, is essential because might contraindicated isolated LT.
ISSN:	0041-1337 1534-6080
DOI:	10.1097/01.tp.0000543982.72180.ff