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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)ResultsDeath
n (% of pregnancies)
Known diagnosis (%) / rate of patients screened for PAVM / CAVMObstetrics eventsRoute of delivery
V D (%) / C S (%)
Goodman et al. 1967
Retrospective
40 / 97
+  80/213 controls
78no statistically significant difference between the 2 groups (HHT and control)NA100 / - / -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
47/161NA11 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
199/484NA13 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
226/560457NANA100 / - / -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 / 24418515 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
NA100 / 13 / 17Miscarriage 20%
Gestational hypertension 2.7%
Preeclampsia 7%
Eclampsia 0.5%
Diabetes 3.2%
PPH 2%
Prematurity 12%
70 / 30
Total599 / 1546720     
  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
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