Retrospective UK multicentre study of the pregnancy outcomes of women with a Fontan repair

Cauldwell, Matthew; Steer, Philip; Bonner, Samantha; Asghar, Omar; Swan, Lorna; Hodson, Kenneth; Head, Catherine; Jakes, Adam D; Walker, Nicola; Simpson, Margaret; Bolger, Aidan P.; Siddiqui, Farah; English, K; Maudlin, Lucy; Abraham, Dilip; Sands, Andrew J; Mohan, Aarthi R.; Curtis, Stephanie; Coats, Louise; Johnson, Mark R.

doi:10.1136/heartjnl-2017-311763

Cited by 38 publications

(24 citation statements)

References 27 publications

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“…Of particular relevance is assessment of exercise capacity. Cyanosis significantly reduces the likelihood of a live birth; oxygen saturation <85% is associated with a less than 12% likelihood of a live birth [167], and the live birth rate may be even lower with cyanosis of this degree in women with a Fontan circulation [173]. Significant right-to-left shunts may be occluded before conception to reduce cyanosis.…”

Section: Contraception and Pregnancy 71 Overviewmentioning

confidence: 99%

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Zentner

Celermajer

Gentles

et al. 2020

Heart, Lung and Circulation

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The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival.

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Section: Contraception and Pregnancy 71 Overviewmentioning

confidence: 99%

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Zentner

Celermajer

Gentles

et al. 2020

Heart, Lung and Circulation

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show abstract

“…Reported miscarriage rates vary according to lesion; being highest (up to 50%) in women with cyanotic heart disease and women with a univentricular (Fontan) circulation 171819. The risk of congenital heart disease in the children of these women is higher than background, with 3% to 5% having the condition 2021.…”

Section: What Are the Risks?mentioning

confidence: 99%

Pregnancy in women with congenital heart disease

Cauldwell

Santos

Steer

et al. 2018

BMJ

Self Cite

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“…1 In a retrospective 10-yr study of women with a Fontan circulation across nine UK congenital cardiac centres 82% of women received prepregnancy counselling. 10 Many women are followed-up from childhood, and receive pregnancy and contraception advice from adolescence. However, women may be lost to follow-up, or have undiagnosed ACHD discovered in pregnancy.…”

Section: Antenatal Management Prepregnancymentioning

confidence: 99%

“…In the Fontan series discussed above, there were 53 live births, of which 12 were spontaneous, 10 had an assisted delivery, and 31 (58.5%) were delivered by CS (12 elective, 19 emergency). 10…”

Section: Cyanotic Diseasementioning

confidence: 99%

Adult congenital heart disease and pregnancy

2018

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There is an increasing number of women with congenital heart disease surviving to childbearing age, with more complex conditions. Women with adult congenital heart disease may tolerate the physiological changes of pregnancy poorly, and can deteriorate at any point, including postpartum. Such women should be counselled of the risks of pregnancy with their condition, and be managed by a specialist multidisciplinary team. Early epidural and a 'low cardiac output' approach allows many women to achieve a normal delivery, but others may require Caesarean section to prevent deterioration in labour. Caution is advised with the commonly used uterotonic drugs. Cardiac disease has remained the leading cause of maternal mortality since the 2000e2002 triennium. Between 2009 and 2014 the deaths of 189 women who died from heart disease associated with, or aggravated by, pregnancy were reported by MBRRACE-UK (Mothers and Babies: Reducing Risk through Physiological changes of pregnancy Pregnancy induces changes in the cardiovascular system to meet the increased metabolic demands of the mother and fetus 5,6 : (i) total blood volume increases by 45% on average; (ii) cardiac output (CO) increases by 30e50%, peaking in the second trimester; (iii) heart rate rises progressively, peaking in Luke Bishop MB ChB FRCA is an advanced trainee in obstetric anaesthesia.

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Retrospective UK multicentre study of the pregnancy outcomes of women with a Fontan repair

Abstract: Women with a Fontan circulation have a high rate of miscarriage and, even if pregnancy progresses to a viable gestational age, a high rate of obstetric and neonatal complications.

Cited by 38 publications

References 27 publications

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Pregnancy in women with congenital heart disease

Adult congenital heart disease and pregnancy

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