Liver Transplantation in Patient with Hereditary Hemorrhagic Telangiectasia or Rendu-Osler-Weber Disease

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Introduction Hereditary hemorrhagic telangiectasia(HHT) is characterized by a disorder in vascular endothelium that presents angiodysplastic lesions in variuos organs. Their presence in the liver can cause serious and possibly mortal complications such as biliary ischemia, portal hypertension or cardiac insufficiency. The only definite cure for HHT with liver involvement is orthotopic liver transplantation(OLT) Materials and Methods 40y old woman is diagnosed with HHT type 2 in 2014. She presents with epistaxis, mucocutaneous telangiectasias, liver arteriovenous malformations(AVM), chronic hyperbilirubinemia and elevated cardiac output(12L/min) with hyperdynamic systolic function and pulmonary hypertension(PH). OLT is suggested as curative treatment. Before OLT her condition worsens and is unresponsive to medical treatment leading to numerous admissions due to decompensated heart failure(HF), GIT bleeding, respiratory infections and hepatic encephalopathy For OLT basic monitorization is carried out and pulmonary artery catheter is inserted before induction. The initial data shows moderate PH and hyperdynamic state. After the general anesthesia induction a transesophagel ultrasound is performed that shows moderate dilation of right cardiac chambers and hyperdynamic contraction function. During the hepatectomy there is moderate bleeding with signs of portal hypertension. During clamping of portal and suprahepatic veins there is no need for vasopressor medication. The hemodynamic changes and metabolic acidosis that occur in reperfusion phase are treated with epinephrine, ephedrine, calcium chloride and sodium bicarbonate. There was no postreperfusion syndrome. In the neohepatic phase norepinephrine infusion(up to 0.2mcg/kg/min) was initiated. The treatment with blood products was guided by ROTEM®. Lactic acid peaked at 8mmol/L and reached 6.9mmol/L at the time of transfer to the ICU.Results and discussion The worst complication in HHT patients with liver involvement is development of HF. That is due to presence of mayor shunts generated by AMV and that augment cardiac output by 25-58%. It is necessary to perform right cardiac catheterization preoperatively to assess pulmonary and right cardiac pressures and to evaluate the shunt and the contractile function. Possible treatments for the cardiopulmonary involvement are embolization or ligature of the hepatic artery, but with serious side effects such as liver necrosis or failure. The OLT should be considered at an early stage in patients with HHT with liver involvement to improve the cardiopulmonary function and avoid the risk for hepatobiliary sepsis and offer a better life quality. Conclusions The OLT is considered to be the only curative treatment for patients with HHT and liver involvement, especially when it is severe. It is important to perform an invasive cardiopulmonary assessment due to high possibility of secondary PH that will dictate the intraoperative anesthetic management.

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