Effective therapy for hepatic M. Osler with systemic hypercirculation by ligation of the hepatic artery and subsequent liver transplantation

Abstract

Hereditary hemorrhagic teleangiectasia, or M. Osler (Osler-Weber-Rendu disease), is an autosomal dominant, systemic fibrovascular dysplasia. This may lead to increased liver blood flow from arteriovenous fistulas. A 45-year-old woman with a known M. Osler was admitted for liver transplantation. On admission, exertional dyspnea was the predominant symptom. Radiological investigations revealed multiple intrahepatic arteriovenous fistulas and consecutive high-output heart failure. Laboratory findings revealed remarkably elevated bilirubin and alkaline phosphatase. To alleviate the high-output cardiac failure, the hepatic artery was ligated. Fourteen months later, the patient presented again with increased levels of bilirubin and recurrent bleeding episodes from esophageal varices grade IV. The patient underwent liver transplantation and post-transplant recovery was excellent. A hyperdynamic circulatory state due to a hepatic M. Osler has been treated in several cases by ligation or embolization of the hepatic artery. This procedure, however, is recommended only for patients with normal liver function and carries a considerably risk of bile duct necrosis.

Hepatic artery embolization for treatment of patients with hereditary hemorrhagic telangiectasia and symptomatic hepatic vascular malformations

“…Of the seven reported patients, one had previously undergone HAE [11]. Another patient required liver transplantation after 14 months because of the development of new collaterals to the hepatic AVMs leading to portal hypertension and recurrent esophageal bleeding [12]. Given the tendency of the hepatic AVMs to draw their blood supply from numerous vessels other than the hepatic artery (such as the pancreato-duodenal vessels, the superior mesenteric, inferior mesenteric, left gastric and splenic arteries), the development of such collaterals with the passage of time after proximal hepatic artery ligation is conceivable.…”

“…b Repeat chest radiograph 2 years following five sessions of HAE; marked reduction in cardiac size. The cardiac output decreased from 13 l/min to 6 l/min subjected to liver transplantation [12,25,[29][30][31][32][33][34][35][36]. Of these patients, one underwent hepatic artery ligation as well as liver transplantation [12], and another patient was subjected to HAE followed by hepatic artery ligation [11]; five patients underwent HAE followed by liver transplantation [25,29,30].…”

“…The least experience exists with hepatic artery ligation [9][10][11][12][13][14]. The reported follow-up periods are short.…”

“…A review of the relevant literature has yielded 7 cases that have been treated with hepatic artery ligation or banding [9][10][11][12][13][14], 25 patients who have undergone HAE [11,[15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30] and 16 patients who have been . 4 a Chest radiograph of a patient with HHT presenting with cardiac failure and intractable abdominal pain.…”

Treatment of High Output Cardiac Failure by Flow-Adapted Hepatic Artery Banding (FHAB) in Patients with Hereditary Hemorrhagic Telangiectasia

“…4,12,14,18,19,24 The treatment of symptomatic intrahepatic HHT includes arterial embolization, ligation (dearterialization), or banding of the proper hepatic artery or a branch of hepatic artery to prevent right heart insufficiency or failure. 2,3,11,18,20,25,26 The only curative therapy in intrahepatic HHT with promising long-term results is liver transplantation. 3,5,11,20,21,23 We report on two cases of symptomatic intrahepatic HHT treated by an intraoperatively ultrasound-controlled gradual banding of the hepatic artery to reduce the high cardiac output improving the clinical conditions for the patients during the waiting time for liver transplantation.…”