Treatment of high output cardiac failure by flow-adapted hepatic artery banding (FHAB) in patients with hereditary hemorrhagic telangiectasia

Abstract

Involvement of abdominal organs in Osler's disease may lead to the development of hepatic arteriovenous shunts with a dilatation of the hepatic artery. Right and subsequent global heart failure due to cardiac valvular insufficiency, pulmonary artery hypertension, and hepatomegaly as well as increased cardiac output may result. This hyperdynamic hepatic blood flow can be reduced by ligature or banding of the hepatic artery or by orthotopic liver transplantation. We report on two female patients suffering from Osler's disease (68 and 76 years old) with severe heart insufficiency (NYHA III-IV) caused by the high hepatic shunt volumes. A gradual banding of the hepatic artery directed by intraoperative flow measurement in the hepatic artery and control of the systemic hemodynamics by Swan-Ganz or COLD catheters was performed in these patients. The banding was achieved by encasing the hepatic artery in a PTFE cuff (length, 1.0 cm). The high cardiac output could be reduced from 11.2 to 7.0 l/min and from 10.7 to 6.0 l/min, respectively. The respective hepatic artery flow was reduced from 2.0 to 0.3 l/min and from 4.0 to 0.7 l/min. An improvement of heart insufficiency, a reduction in the severity of the cardiac valvular insufficiency, and a reduction of the pulmonary arterial hypertension could be already observed intraoperatively. One patient died of right cardiac failure after an orthotopic liver transplantation 7 months later. The other one died 3 years after the banding. The banding of the hepatic artery controlled by hepatic arterial flow measurement can be considered as an effective and safe palliative procedure in intrahepatic HHT compared to therapeutic alternatives such as hepatic artery ligation or embolization.