HHT International Guidelines Pregnancy Checklist.pdf

The HHT Guidelines provide recommendations for care based on evidence and expertise from HHT experts worldwide, aiming to ensure people with HHT receive the best care possible. For pregnant women with HHT, the guidelines recommend assessment for risk of pregnancy and delivery complications, access to a multidisciplinary maternal-fetal medicine team, and screening for pulmonary AVMs and brain VMs. Specific recommendations include:

Pre-pregnancy consultation with an obstetrician to discuss genetic diagnosis options
Screening for pulmonary AVMs using agitated saline transthoracic contrast echocardiography or low-dose non-contrast chest CT in asymptomatic patients
Diagnostic testing for pulmonary AVMs using low-dose non-contrast chest CT in symptomatic patients
Treatment of pulmonary AVMs starting in the second trimester
MRI without gadolinium in second trimester for symptomatic patients with brain VMs
Management at a tertiary care center by a multidisciplinary team for women with untreated pulmonary AVMs or brain VMs
Epidural anesthesia not withheld due to HHT diagnosis, and no requirement for screening for spinal vascular malformations
Vaginal delivery allowed for women with known, non-high risk brain VMs, with consideration for assisted second stage on a case-by-case basis.