Abstract
INTRODUCTION AND IMPORTANCE: Arteriovenous malformations (AVMs) in the liver caused by hereditary hemorrhagic telangiectasia (HHT) influence pulmonary artery hypertension (PAH). Liver transplantation (LT) is the most common treatment for HHT-induced hepatic AVMs. However, LT is contraindicated for patients with severe PAH. There is controversy regarding the ideal therapeutic approach for HHT with PAH and hepatic AVMs. CASE PRESENTATION: We present the case of a 48-year-old female with PAH and HHT. After the initiation of PAH-targeted drugs, we considered that the PAH was mainly caused by high cardiac output secondary to multiple diffuse AVMs in the liver. LT was contraindicated due to high mean pulmonary arterial pressure (mPAP), and we opted to perform transcatheter embolization as an alternative treatment for the AVM. Multiple-stage embolization sessions did not effectively improve the shunt in the liver or the pulmonary hemodynamics. The patient died of an uncontrolled gastrointestinal hemorrhage. CLINICAL DISCUSSION: LT was considered in our case; it was contraindicated because of pulmonary hypertension that was in line with the model for end-stage liver disease exception criteria. Repeated embolization did not reduce the liver shunt or improve pulmonary hemodynamics, possibly due to the diffuse distribution of AVMs in the liver and the rapid development of new collateral vessels with each embolization. Recently, pulmonary vascular resistance (PVR) has been proposed as a more appropriate index for stratifying perioperative risk. CONCLUSION: Based on previous reports and our experience, rapid decision-making regarding LT may be needed based on mPAP and PVR after the initiation of PAH-targeted drugs.