Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia

To review the effects of hereditary hemorrhagic telangiectasia (HHT) in the parturient and the anesthetic management of such patients during pregnancy and delivery.

A literature search (1966-2008) was performed using Medline and EMBASE databases. Bibliographies of retrieved articles were searched for additional sources.

Hereditary hemorrhagic telangiectasia affects 1 in 5000-8000 people. It is a genetic condition in which vascular dysplasia affects many organs particularly the pulmonary, cerebral, gastrointestinal, and spinal vasculature. A large proportion of women with HHT have uneventful pregnancies. However, women can present in pregnancy with clinically silent but potentially life-threatening features of the disorder including fatal hemorrhage from ruptured arteriovenous malformations (AVMs), systemic emboli, and high output cardiac failure secondary to arteriovenous shunting. Literature on the anesthetic management of HHT in pregnancy is limited. Both general and regional anesthetic techniques have been successfully performed in these patients, but are reliant on identifying the presence of specific AVMs; avoidance of cardiovascular instability; and prophylaxis against systemic emboli secondary to pulmonary AVM shunting. The presence of spinal AVMs is considered a relative contraindication to regional techniques. As with other systemic AVMs, these can develop and increase in size during pregnancy with implications for the timing of screening and surveillance.

An understanding of the presence and potential development of life-threatening AVMs during pregnancy is imperative for anesthesiologists caring for parturients with HHT. Even in the asymptomatic patient, a high index of suspicion should be maintained, screening performed where possible and anesthetic technique adapted accordingly.