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Table 1 Characteristics of pregnancies in women with HHT: A summary of 5 case series published between 1967 and 2014

From: Hereditary haemorrhagic telangiectasia and pregnancy: a review of the literature

Study

(Author, type)

Women (n) / Pregnancies (n)

Live births (n)

Results

Death

n (% of pregnancies)

Known diagnosis (%) / rate of patients screened for PAVM / CAVM

Obstetrics events

Route of delivery

V D (%) / C S (%)

Goodman et al. 1967

Retrospective

40 / 97

+  80/213 controls

78

no statistically significant difference between the 2 groups (HHT and control)

NA

100 / - / -

Miscarriage 14.4 vs 10%

Abnormal outcome 50 vs 42%

Prematurity 9.4 vs 10.3%

Stillbirth 3.1 vs 2.6%

NA

Shovlin et al. 1995

Retrospective

47/161

NA

11 severe events (6.8% of pregnancies)

PAVM shunt deterioration n = 6

Cerebro vascular accident n = 3

Fatal Pulmonary hemorrhage n = 2

10/11 events in the « PAVM + » group

3 (1.9)

100 / - / -

No Significant difference

in the Miscarriage rate between the « PAVM + » and « PAVM – » group

NA

Shovlin et al. 2008

Prospective+retrospective

199/484

NA

13 severe events (2.7% of pregnancies)

Myocardial infarction n = 1 (died)

Cerebro vascular accident n = 6

(2 died / 2 ischaemic, 3 haemorrhagic, 1 NA)

Pulmonary hemorrhage n = 6

(2 died)

5 (1)

26 / - / -

 

NA

Wain et al. 2012

Retrospective

226/560

457

NA

NA

100 / - / -

T1 Miscarriage 14.3%

Preeclampsia 0.6%

PPH 6.2%

Prematurity 13.8% LBW 10.7%

73.5 / 20.6

De Gussem et al. 2014

Retrospective

87 / 244

185

15 severe events (6.1% of pregnancies)

Heart failure n = 1

Hemothorax n = 4

(4 undiagnosed PAVMS)

Hemoptysis n = 2

Transient Ischaemic attack n = 2

Post partum: Deep veinous thrombosis n = 1

Pulmonary embolism n = 1

Myocardial ischemia n = 2

Intracranial hemorrhage n = 1

Hemothorax n = 1

NA

100 / 13 / 17

Miscarriage 20%

Gestational hypertension 2.7%

Preeclampsia 7%

Eclampsia 0.5%

Diabetes 3.2%

PPH 2%

Prematurity 12%

70 / 30

Total

599 / 1546

720

     
  1. Legends: VD Vaginal delivery, CS Cesarean section, PAVM Pulmonary arteriovenous malformation, CAVM Cerebral arteriovenous malformation, LB Live birth, PP Post partum, PPH Post partum hemorrhage, LBW Low-birth weight, NA Data Not Available