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

Abstract

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

Complications and clinical outcome of hepatic artery embolisation in patients with hereditary haemorrhagic telangiectasia

“…Our procedure-related complication rate of 20 % compares favourably with the 60 % complication rate reported by Lerut and colleagues [11] as well as with the 54 % (7 of 13 patients) experienced by Dupuis-Girod [13]. Six of the documented 35 patients in Lerut’s cohort required additional surgery [11] as was the case in 4 of our 20 patients.…”

“…Similarly, of the six transplanted patients reported by Azoulay and colleagues, two died [12]. As opposed to this, Dupuis-Girod reported a mortality of 8 % in 13 of their 753 patients (1.7 %) who underwent liver transplantation [13]. …”

Natural History and Outcome of Hepatic Vascular Malformations in a Large Cohort of Patients with Hereditary Hemorrhagic Teleangiectasia

“…Complications included the following: HOCF (with fatigue, dyspnea associated with ascites and/or limb edema; all patients were classified according to the New York Heart Association [NYHA] functional classification), complicated portal hypertension (with ascites and/or gastrointestinal bleeding), encephalopathy, cholangitis and mesenteric angina [25, 26, 32, 34, 35]. The etiology of ascites (whether derived from HOCF or portal hypertension) was established on the basis of clinical, laboratory and instrumental data.…”

Embolisation of PAVMs reported to improve nosebleeds by a subgroup of patients with hereditary haemorrhagic telangiectasia

“…Since the shared common feature of all systemic AVMs, PAVMs and iron deficiency anaemia is a higher cardiac output that reduces after AVM/anaemia treatment [9, 11–13], we suspect this may underlie the reported improvements, particularly for larger AVMs. Potential flow, vasodilatory and other mechanisms deserve evaluation in further studies, which could employ the validated epistaxis severity scoring system [14], explore whether particular subgroups are more likely to report epistaxis improvement following PAVM embolisation and evaluate whether epistaxis treatment requirements reduce following PAVM embolisation.…”