Management of Fontan circulation in pregnancy: a multidisciplinary approach to care

Wolfe, Natasha K.; Sabol, Bethany; Kelly, Jeannie C.; Dombrowski, Michael; Benhardt, Amber C.; Fleckenstein, Jaquelyn; Stout, Molly J.; Lindley, Kathryn J.

doi:10.1016/j.ajogmf.2020.100257

Cited by 13 publications

(46 citation statements)

References 19 publications

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“…A right heart catheterization is typically performed approximately every 10 years in a patient with Fontan circulation [7,14]. If haemodynamics have not been assessed in the past 5 years prior to conception, it may be considered in order to evaluate Fontan pressures, pulmonary artery pressures, PVR and to assess for the presence of right to left shunts such as fenestrations or collaterals [20].…”

Section: Preconception Counsellingmentioning

confidence: 99%

“…Causes of premature delivery can vary from premature rupture of membranes (6%), antepartum haemorrhage (10%) and intrauterine growth restriction (IUGR, 31%) [8,40]. Given the high risk of foetal complications including stillbirth and IUGR, timing for an earlier delivery, approximately 37 weeks, and consultation with neonatology can be considered [20].…”

Section: Preconception Counsellingmentioning

confidence: 99%

“…Although single ventricle patients are preload dependent, Valsalva is not absolutely contraindicated. An assisted second stage delivery with forceps or vacuum extraction may be considered to reduce the duration of Valsalva [5,20].…”

Section: Deliverymentioning

confidence: 99%

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Management of the Fontan patient during pregnancy

Gupta¹,

Thompson

Lindley

2023

Current Opinion in Cardiology

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Purpose of reviewPatients with single-ventricle Fontan palliation surgery often wish to pursue pregnancy. Pregnancies should be planned with well tolerated and effective contraception, and preconception risk stratification by adult congenital heart disease and maternal foetal medicine specialists.Recent findingsAlthough infertility and foetal complications, including pregnancy loss, preterm birth and foetal growth restriction, are common, most patients with Fontan palliations can successfully complete pregnancy with a team-based approach. Important risk predictors are resting oxygen saturations, baseline functional status and the presence of systemic complications of the Fontan repair, including advanced Fontan associated liver disease, plastic bronchitis and ventricular dysfunction. Common maternal cardiovascular complications include arrhythmia, heart failure and thromboembolism. Delivery planning with input from an obstetric anaesthesiologist who has knowledge of complex congenital heart defects can facilitate appropriate, individualized monitoring and pain control. A vaginal delivery with consideration of an assisted second stage is appropriate for most single ventricle patients, in the absence of obstetric or foetal indications for caesarean delivery. Close postpartum monitoring and follow up is recommended, as the early postpartum period is the highest risk time for cardiovascular complications in patients with congenital heart disease.SummaryA multidisciplinary approach to managing pregnancy and delivery in patients with Fontan circulation facilitates optimal maternal and infant outcomes.

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Section: Preconception Counsellingmentioning

confidence: 99%

Section: Preconception Counsellingmentioning

confidence: 99%

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Management of the Fontan patient during pregnancy

Gupta¹,

Thompson

Lindley

2023

Current Opinion in Cardiology

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“…An individualized approach in pre-conception considerations should also be used in the context of FALD because the risk is undoubtedly higher in patients with hepatic fibrosis or cirrhosis than in those with mild liver disease. 49 According to the Chinese study on pregnant women with concomitant cirrhosis, severe maternal complications including placenta abruption, postpartum hemorrhage >1,000 ml, hysterectomy, multiorgan failure, and upper gastrointestinal bleeding were present in as many as 32% of cases. Also, conception itself is uncommon in the setting of cirrhosis because of endocrinal disturbances, particularly in estrogen metabolism.…”

Section: Pregnancy In Faldmentioning

confidence: 99%

“…If present, the management options include pharmacotherapy with nonselective β-blockers throughout the pregnancy or invasive treatment in the case of advanced-stage disease (preferably during the second trimester). 49 Importantly, pregnant women with Fontan circulation have the highest risk of antenatal and peripartum bleeding in patients with congenital heart disease, and the overlapping FALD-associated coagulopathy may exacerbate this complication. 50,52…”

Section: Pregnancy In Faldmentioning

confidence: 99%

Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners

Ciepłucha

Budts

Gewillig

et al. 2022

US Cardiology Review

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Nowadays most patients with a univentricular heart after Fontan repair survive until adulthood. One of the hallmarks of Fontan circulation is permanently elevated central venous pressure, which leads to congestive hepatopathy. Subsequently, liver fibrosis, cirrhosis, or hepatocellular carcinoma may occur, all of them constituting an entity called Fontan-associated liver disease (FALD). Given that these complications convey poor prognosis, the need for life-long hepatic surveillance is not in doubt. Many serum biomarkers and sophisticated imaging techniques have been proposed to avoid invasive liver biopsy in this cohort, but none proved to be a relevant surrogate of liver fibrosis seen in histopathological specimens. The surveillance models proposed to date require an extensive diagnostic work-up, which can be problematic, particularly in resource-depleted countries. Moreover, the question of combined heart–liver transplant is gaining more attention in the Fontan cohort. The aim of this study is to provide practical information on the pathophysiology of FALD and to propose a simplified framework for the routine assessment of liver status in Fontan patients that would be helpful in the decision-making process.

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Congenital Heart Disease in Adults

John,

Adebo

2024

Board Review Atlas of Pediatric Cardiology

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Management of Fontan circulation in pregnancy: a multidisciplinary approach to care

Cited by 13 publications

References 19 publications

Management of the Fontan patient during pregnancy

Management of the Fontan patient during pregnancy

Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners

Congenital Heart Disease in Adults

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