Effects of pregnancy on hereditary haemorrhagic telangiectasia (HHT)

Introduction Due to visceral arteriovenous malformations (AVM) and recurrent bleeding, pregnancies in patients with hereditary haemorrhagic telangiectasia (HHT) are classified as high-risk pregnancies. The aim of this case series was to analyse the development of epistaxis and the systemic complications before, during, and after pregnancies in HHT patients.

Methods Patients presented in our HHT-centre after their pregnancy. Symptoms and complications before, during, and after pregnancy were documented using the epistaxis severity score (ESS) to record the intensity of epistaxis.

Results All patients reported aggravation of epistaxis during their pregnancies concerning duration as well as intensity (ESS before pregnancy (m ± SD): 3.7 ± 1.1; during pregnancy (m ± SD): 5.7 ± 1.5). The ESS remained elevated in all patients after delivery compared to the score before pregnancy ((m ± SD): 4.6 ± 1.4 at follow up of 15, 26 and 42 months respectively). Two patients underwent screening for pulmonary AVM (PAVM) prior to their first pregnancy reporting no complications. The third patient had an ischemic stroke (week 22) and was diagnosed with persisting foramen ovale and PAVM in the following examinations. Initial diagnosis of HHT was made at this point, and the lesions were treated interventionally.

Discussion Complications occur rarely in spite of the high-risk status of pregnancies, but can be severe and are often caused by bleeding. Therefore, screening for PAVM and (if indicated) embolization is strongly recommended to minimize the risk for severe complications such as haemoptysis and strokes due to paradoxical emboli.

Poster-PDF A-1555.pdf

Conflict of interest

Der Erstautor gibt keinen Interessenskonflikt an.

Publication History

Article published online:
13 May 2021