Hereditary hemorrhagic telangiectasia, liver vascular malformations and cardiac consequences

Cardiopulmonary Exercise Testing Demonstrates Maintenance of Exercise Capacity in Patients With Hypoxemia and Pulmonary Arteriovenous Malformations

“…These findings have not been followed-up, due to a recent focus on pulmonary hypertension in the field, 12,[19][20][21][22][23][24][25][26][27] and in terms of prophylaxis.…”

“…12 When pulmonary hypertension does occur, 19,20 this results not from hypoxia, but from other pathophysiological processes, particularly pulmonary arterial hypertension, 12,[21][22][23][24] and pulmonary venous hypertension associated with hepatic AVMs and high outputs states. 12,[25][26][27] Our hypothesis was that in the presence of hypoxemia but absence of pulmonary hypertension, exercise capacity can be maintained by cardiovascular andhematological 28 adaptations. The goal of the current study was to draw attention to the clinical differences between hypoxemic patients with PAVMs and other more common conditions by further elucidating the mechanisms behind the generally good exercise tolerance in PAVM patients.…”

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

“…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]. Therapeutic approaches toward symptomatic intrahepatic shunts depends on type of shunt and associated symptoms [1,7].…”

Hereditary haemorrhagic telangiectasia

“…Hepatic AVMs (HAVMs) occur in 32-84% of HHT patients, being more common in HHT2, but are only symptomatic in approximately 8% of cases (13,25,(54)(55)(56) . The most common complications are high output cardiac failure, portal hypertension and biliary necrosis (55) .…”

“…The most common complications are high output cardiac failure, portal hypertension and biliary necrosis (55) . Screening, with Doppler ultrasound (US), is not recommended unless patients are symptomatic or have elevated liver function tests (25,56) .…”