P- 18 TREATMENT WITH BEVACIZUMAB IN HIGH OUTPUT CARDIAC FAILURE DUE TO SEVERE HEPATIC COMPROMISE IN HEREDITARY HEMORRHAGIC TELANGIECTASIA PATIENTS: OBSERVATIONAL COHORT STUDY

Introduction and Objectives

Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant vascular dysplasia affecting 1/5000 individuals. Epistaxis, mucocutaneous telangiectasias and vascular malformations affecting internal organs (brain, lungs, liver and digestive tract) are hallmarks of HHT. Though liver involvement occurs in 80% of patients, including abnormal portal-venous, arterio-portal, and arterio-venous shunts, overt clinic is only present in 8% and may present as secondary high-output cardiac failure (HOCF), portosystemic encephalopathy, noncirrhotic portal hypertension and/or bile duct ischemia. This study aimed to report a single HHT Reference Center experience in the antiangiogenic treatment with bevacizumab (anti VEGF-Vascular Endothelial Growth Factor) for treating adult HHT patients with HOCF due to severe liver disease.

Materials and Methods

Observational cohort study. Baseline clinical/analytical characteristics were recorded and echocardiographic values for cardiac index in L/min/m² and cardiac output in L/min (CI and CO) before and after bevacizumab treatment were compared when available with a paired signed rank test.

Results

Thirteen patients were included from July/2013-June/to 2022, nine were women and the median age was 68 [IQR: 53-71]. All of them with HOCF; eleven had edema/ascites and six also had refractory iron deficiency anemia. Regarding liver compromise: nine had hepatomegalia, all had diffuse telangiectasias, six portal-venous, ten arterio-venous and eight arterio-portal shunts, while only two had ischemic bile duct injury. Basal median CI was 4.1 [3.8-4.8] and CO was 7.5 [6.1-8.6] (n=11). Median Bevacizumab number of the received doses was 6 [4-6]. At least one post-treatment result during the first year of treatment was available in 8 patients with a median CI of 3.5 [3.1-4.3] (p<0.05) and CO of 6.2 [4.5-3.0] (p<0.05). Two patients received liver transplantation while on treatment.

Conclusions

These results supporting bevacizumab treatment in HHT patients with severe liver disease are in line with previous reports.