The Role of Liver Transplantation in the Treatment of Hereditary Hemorrhagic Telangiectasia: A Short Literature Review

Scelzo, Chiara; Greco, Stefania; Bonanni, L.; Cocco, Pierpaolo Di; D’Angelo, M.; Laurenzi, Chiara; Pisani, F.; Famulari, A.; Orlando, Giuseppe

doi:10.1016/j.transproceed.2007.05.022

Cited by 17 publications

(20 citation statements)

References 17 publications

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“…Thus, sub-selective hepatic artery embolisation is reserved only for highly selected patients who have failed medical therapy and who are not considered candidates for liver transplantation [42,47]. Liver transplantation has been used for highly symptomatic individuals with high-output heart failure due to extensive liver VMs [51,52]. Excellent success in reducing cardiac output and alleviating symptoms has been reported in some patients.…”

Section: Treatmentmentioning

confidence: 99%

The pulmonary vascular complications of hereditary haemorrhagic telangiectasia

Faughnan¹,

Granton²,

Young³

2009

Eur Respir J

124

116

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Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder, characterised by the presence of vascular malformations. The pulmonary vascular complications of HHT include pulmonary arteriovenous malformations, pulmonary hypertension associated with high-output heart failure and liver vascular malformations and, finally, pulmonary arterial hypertension secondary to HHT. In the present review, the authors describe the clinical presentation, diagnosis and management of all three pulmonary vascular presentations of HHT, as well as the underlying genetics and pathophysiology.

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Section: Treatmentmentioning

confidence: 99%

The pulmonary vascular complications of hereditary haemorrhagic telangiectasia

Faughnan¹,

Granton²,

Young³

2009

Eur Respir J

124

116

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“…Mutations in the genes for endoglin (ENG) and for activin A receptor type II-like kinase 1 (ALK-1), located on chromosomes 9 and 12, respectively, have been associated with HHT [2,3]. The homozygous state appears to be lethal [4].…”

Section: Introductionmentioning

confidence: 99%

“…However, the abnormalities appear later and tend to be milder. The genetic abnormality involves a mutation in the gene for ALK-1 [2].…”

Section: Introductionmentioning

confidence: 99%

“…Liver involvement has been reported in up to 31% of the affected patients [2,4,7], predominantly in HHT type 2. However, radiological signs of liver involvement are detectable in up to 74% of patients and vary according to the radiological technique used.…”

Section: Introductionmentioning

confidence: 99%

“…Interestingly, recurrent arteriovenous malformations have been reported up to ten years after liver transplantation [2,11].…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Hereditary Hemorrhagic Telangiectasia of the Liver Complicated by Ischemic Bile Duct Necrosis and Sepsis: Case Report and Review of the Literature

et al. 2009

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Long-term outcome of patients with hereditary hemorrhagic telangiectasia and severe hepatic involvement after orthotopic liver transplantation: A single-center study

Dupuis‐Girod

Chesnais²,

Ginon

et al. 2010

Liver Transpl

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Hepatic involvement occurs in up to 74% of patients with hereditary hemorrhagic telangiectasia (HHT) and is characterized by a spectrum of arteriovenous malformations. Three different types of intrahepatic shunting may be present: hepatic artery to hepatic veins, hepatic artery to portal vein, and portal vein to hepatic vein. Hepatic involvement in HHT may lead to biliary ischemia, portal hypertension, or high-output cardiac failure (HOCF). Orthotopic liver transplantation (OLT) has been proposed as the only definitive curative treatment. The aim of this study was to evaluate the long-term outcome of patients with hepatic involvement due to HHT after OLT with respect to mortality, cardiac and hepatic status, epistaxis, and quality of life. Patients with HHT and severe hepatic vascular malformations who underwent OLT in the Lyon Liver Transplant Unit (LLTU) from 1993 to 2007 were followed at the LLTU and the French Reference Center for HHT. Quality of life was evaluated with the Short Form 36 questionnaire. There were 13 patients who fulfilled the entry criteria of the study (12 women and 1 man). The mean age at the time of OLT was 51.8 years (range ¼ 33-65 years). Indications for OLT were cardiac failure (n ¼ 9), biliary necrosis (n ¼ 2), both cardiac failure and biliary necrosis (n ¼ 1), and hemobilia (n ¼ 1). The mean duration of follow-up was 109 months (range ¼ 1-200 months). Twelve patients (92.3%) are still alive. For the 9 patients with HOCF, the mean cardiac index decreased from 5.4 L/minute/m 2 before OLT to 3.0 L/minute/m 2 after OLT. No severe hepatic complications were observed after OLT. Nine of the surviving patients (75%) experienced dramatic improvements in epistaxis and quality of life, including an

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The Role of Liver Transplantation in the Treatment of Hereditary Hemorrhagic Telangiectasia: A Short Literature Review

Cited by 17 publications

References 17 publications

The pulmonary vascular complications of hereditary haemorrhagic telangiectasia

The pulmonary vascular complications of hereditary haemorrhagic telangiectasia

Hereditary Hemorrhagic Telangiectasia of the Liver Complicated by Ischemic Bile Duct Necrosis and Sepsis: Case Report and Review of the Literature

Long-term outcome of patients with hereditary hemorrhagic telangiectasia and severe hepatic involvement after orthotopic liver transplantation: A single-center study

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