A Case of too Much Love from the Liver: Hereditary Hemorrhagic Telangiectasia with Liver Vascular Malformation Presenting with High-output Heart Failure

Purpose: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant syndrome that can cause visceral vascular malformations. Hepatic involvement may lead to portal hypertension and high-output cardiac failure. A 48-year-old female with HHT presented with worsening shortness of breath/lower extremity edema over a 6-month period and was found to have heart failure with high cardiac output of 8.2 L/min. She was diagnosed with HHT at the age of 21 years based on the presence of frequent epistaxis, telangiectasias on lips and tongue and family history of HHT in her mother and brother. She had a prior CT of the chest and brain with no evidence for arterio-venous malformations (AVMs) and she had no previous history of upper or lower gastrointestinal bleeds. A CT scan of the abdomen at our institution showed markedly dilated portal vein, hepatic vein and inferior vena cava with heterogeneous enlarged liver and innumerable hyperdense nodules. Vascular US of the liver showed high velocity and low resistive indicies of the hepatic arteries with multiple hepatic intraparenchymal shunts. A diagnosis of liver AVMs causing high-output heart failure was made. Due to failure of intensive medical therapy, a liver transplantation was considered but the patient was not a candidate because of poor overall condition and the presence of severe pulmonary hypertension. Therefore, hepatic artery embolization was done to temporize and reduce heart failure symptoms with partial improvement in her symptoms and oxygen requirements. Given the known transient effect of embolization and the recurrence of symptoms in most patients, bevacizumab (monoclonal antibody against VEGF) was used to try to decrease shunting through hepatic AVMs based on previous literature. After her first dose of bevacizumab, the patient developed oropharyngeal bleeding that rapidly progressed to frank uncontrolled bleeding leading to aspiration of blood and culminating in cardiac arrest and death. This case illustrates that liver AVMs in HHT can cause increased preload leading to high-output heart failure. Bevacizumab should be used with caution in these patients due to increased risk for hemorrhage.