Liver transplantation for cardiac failure in patients with hereditary hemorrhagic telangiectasia

Abstract

Liver involvement in hereditary hemorrhagic telangiectasia may lead to high-output cardiac failure. Few data have been reported on orthotopic liver transplantation (OLT) for these patients. In this paper, we describe two patients treated by OLT as a salvage procedure for cardiac failure, and we review literature on this subject. Our two patients resumed normal cardiac function after OLT. This procedure appears to be a promising therapy with good longterm results despite dissection difficulties encountered due to the collateral arterial network reorganization. (Liver Transpl 2005;11:834-838.)

Hereditary Hemorrhagic Telangiectasia of the Liver Complicated by Ischemic Bile Duct Necrosis and Sepsis: Case Report and Review of the Literature

“…Liver involvement in HHT has been described in multiple case reports [7,[12][13][14] and case series [8][9][10]. As Fig.…”

Liver Transplantation for High Output Heart Failure Secondary to HHT: A Case Report and Review of the Literature

“…High output cardiac failure is the most common clinical presentation in patients with hepatic involvement [5,8,10]. Shunting of blood from the hepatic artery to the hepatic vein results in a hyperdynamic circulation and leads to high output heart failure [4,7,8,11].…”

“…Patients with heart failure are initially treated with conventional medical therapy such as diuretics and beta blockers [7]. In the past, ligation of the hepatic artery or shunt embolization was attempted [1,5,8,10]. Although amelioration or resolution of symptoms has been reported in most of the cases, this effect is generally only transient and treatment maybe associated with significant morbidity and mortality, mostly in the form of biliary and/or hepatic necrosis [8,10].…”

Successful liver transplantation for Rendu–Weber–Osler disease, a single centre experience

“…In the six cases described, two cavojugular and four portocavojugular bypasses were performed. Thevenot et al [7] also described a case report with the use of 25 blood units while a TPCS was necessary in emergency for a spontaneous rupture of the spleen after portal clamping. In Boillot et al series [30], one LTx for high CO and biliary symptoms required 26 blood units and a VVPB was used.…”

“…In these conditions, liver transplantation (LTx) is the only effective therapy option. The two other indications of LTx for patients with HHT are heart failure due to high cardiac output (CO) or portal hypertension because of hemodynamic disturbances throughout intrahepatic shunts [5,[7][8][9][10]. However, according to the experience of certain centres, LTx was revealed to be difficult due to hemostasis concerns requiring greater blood transfusion need [11].…”