P-129 liver transplantation in patients with hereditary hemorrhagic telangiectasia. experience of two cases at an argentine hht referral center

Introduction and Objectives: This study aimed to describe two cases of liver transplantation in HHT with severe hepatic involvement. Materials and Methods: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease characterized by mucocutaneous bleeding telangiectasias and arteriovenous malformations in organs, including the lungs, central nervous system, liver and gastrointestinal tract. Hepatic involvement occurs in 78% of patients, of which 8% develop relevant clinical manifestations. Severe liver vascular malformations may lead to high-output cardiac failure with pulmonary hypertension, portal hypertension, or intrahepatic biliary ischemia. Although anti-angiogenic treatment with bevacizumab can improve symptoms, liver transplantation (LT) emerges as a definitive treatment with Improvement in cardiac function. Liver transplant patients' timing and proper selection are crucial and represent a challenge. We report two cases of liver transplantation in HHT with severe hepatic involvement. Results: Patient 1 (P1) was a 47-year-old male, and patient 2 (P2) was a 37-year-old female with multiple arteriovenous malformations (AVMs). Cardiac index (CI) and cardiac output were 6.8, 9.5 (L/min/m2), and 5.8, 9.3(L/min) in P1 and P2, respectively, associated with dilated cardiomyopathy with mean pulmonary hypertension of 60 mmHg in P1 and 33 mmHg in P2. Additionally, both presented portal hypertension and ischemic biliopathy refractory to medical treatment. They also received bevacizumab one year before LT, showing marked clinical improvement. P1 was anticoagulated due to a mechanical aortic valve. After intensive diuretic therapy, the mean pulmonary pressure was 35 mmHg in P1. Natural MELD was 11 in both patients, and additional MELD was 26 and 28, respectively. Six hours of orthotopic liver transplant with cava preservation and a high-quality donor were performed. Only 1 and 3 units of red cells were transfused, respectively, with non-hemorrhagic perioperative events observed. Post-transplant complications in P1 included vasoplegic shock, splenic steal (solved with artery embolization), and reversible renal failure due to hypotension and tacrolimus, while P2 presented a mild reversible cellular rejection. Tacrolimus was prescribed for both cases due to its antiangiogenic properties. Pre LT clinical characteristics are shown in Table 1. Pre LT and Post LT hemodynamic and echocardiographic parameters are shown in Table 2. After 56 months, P1 is in good clinical conditions CI of 5.6 (L/min/m2), and low doses of diuretic requirements. P2 was discharged ten days post-transplantation and, after 43 months, is in an excellent performance with a CI of 3.31 (L/min/m2). Conclusions: We provide data about the applicability and timing of liver transplantation in selected patients with HHT with severe hepatic involvement. According to our knowledge, this is the first Latin American report of liver transplantation in HHT. Despite the high risk of bleeding, highlight the low rate of perioperative transfusion requirements in both cases.