Anaesthetic management of parturients with hereditary haemorrhagic telangiectasia for caesarean section

High Output Cardiac Failure in a Parturient with Hereditary Haemorrhagic Telangiectasia

“…Central neuraxial block has the advantage of reducing afterload and preload but is not well described in these patients. The incidence of spinal AVMs is up to 1 to 2% 1 , and these should be excluded by magnetic resonance imaging before neuraxial anaesthesia is performed 3,6 . In this patient spinal magnetic resonance imaging had never been performed.…”

“…ultimately, orthotopic liver transplantation may be required for severe hepatic AVMs 12,13 . In pregnancy there is less published experience with managing these patients and most literature deals with patients with pulmonary AVMs [2][3][4][5][6] . It is thought that the cardiac failure associated with hepatic AVMs is reversible 14 so it has been recommended that these patients be delivered as soon as feasible 7 .…”

“…Hereditary haemorrhagic telangiectasia (HHT), or Osler-Rendu-Weber disease, is an autosomal dominant genetic condition that affects one in 5000 to 8000 1 . Although most literature on HHT and pregnancy deals primarily with pulmonary arteriovenous malformations (AVM), which cause significant morbidity and mortality [2][3][4][5][6] , HHT affects the liver in 41 to 78% of cases 7 . Most patients are asymptomatic, especially prior to pregnancy, but with the added oxygen demands of pregnancy they may present with high output cardiac failure, or less commonly with portal hypertension, biliary disease, abnormal liver function tests, encephalopathy and parenchymal abnormalities.…”

Perioperative Management of a Patient with Hereditary Hemorrhagic Telangiectasia and Deep Vein Thrombosis: A Case Report

“…Spinal AVMs are found in less than 1% of HHT patients [4]. El Shobary et al reported a case of cesarean section performed under spinal anesthesia in a pregnant woman with HHT after excluding the presence of spinal AVM by head and spinal MRI; the authors stated that evaluation for spinal AVM is necessary when considering neuraxial anesthesia in HHT patients [14]. On the other hand, Gussem and colleagues successfully performed spinal or epidural anesthesia for 92 deliveries in HHT women without prior screening for spinal AVMs [13], and they suggested that screening for the presence of spinal AVMs is not routinely recommended in pregnant women with HHT.…”

Cesárea en una embarazada con telangiectasia hemorrágica hereditaria: a propósito de un caso

“…Las gestantes con HHT presentan un empeoramiento de su enfermedad debido a los cambios hemodinámicos fisiológicos que produce el propio embarazo, con un importante aumento de la morbimortalidad durante este período (5). Es importante el manejo multidisciplinario de estas pacientes durante el embarazo, así como la elaboración de un plan anestésico para el momento del parto.…”