Reproductive Issues in Patients With the Fontan Operation

Wichert-Schmitt, Barbara; D’Souza, Rohan; Silversides, Candice K.

doi:10.1016/j.cjca.2022.04.020

Cited by 12 publications

(5 citation statements)

References 69 publications

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“…27 Subsequent deficiency in estrogen and testosterone production may be a potential explanation for the high frequency of hypogonadism seen in Fontan populations. 5,6 A significant positive association was demonstrated between nTPV and age in all subjects, with no difference in this relationship between Fontan and Control groups. This may suggest a "normal" age related phenomenon.…”

Section: Discussionmentioning

confidence: 74%

“…Hypothalamic-pituitary-axis (HPA) dysfunction may be more common than realized in post Fontan circulation given the high frequency of short stature, central hypothyroidism and hypogonadic hypogonadism reported in this unique patient population. [3][4][5][6] One previous study has demonstrated significantly larger pituitary volumes in children post Fontan compared to controls, however the sample size was small, and methodology did not allow segmental volumetrics. To date these findings have not yet been replicated.…”

Section: Introductionmentioning

confidence: 99%

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Segmental MRI Pituitary and Hypothalamus Volumes post Fontan: An analysis of the Australian and New Zealand Fontan Registry

Gee,

Yang,

Gentles

et al. 2024

Preprint

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ObjectiveShort stature, central hypothyroidism and infertility are common in those with a Fontan circulation. Given that the Fontan circulation often results in hepatic portal venous congestion, we hypothesize that the hypothalamic-pituitary portal circulation is also affected, contributing to subsequent hypothalamic-pituitary axis dysfunction.MethodsMRI data from the Australian and New Zealand Fontan Registry (86 cases) was compared to 86 age- and sex- matched normal published controls. Total pituitary volumes (both anterior and posterior glands) were measured using a manual tracing segmentation method, and hypothalamic (and subunit) volumes using an automated segmentation tool. Measured gland volume was normalized to total brain volumes. A generalized linear model was used for statistical analysis.ResultsNormalized total pituitary volumes (nTPV) were increased in Fontan patients compared to controls (p<0.0001), due to an increase in anterior pituitary volumes (nAPV) (p<0.0001), with no difference in normalized posterior pituitary volumes (p= 0.7). Furthermore, normalized anterior and tubular hypothalamic subunit groups) were increased in Fontan patients compared to the controls (p<0.01 andp<0.0001, respectively).The time between Fontan and MRI was positively related to nTPV, nAPV and bilateral hypothalamic volumes. nTPV increased with age, and the increase in nAPV was greater in Fontan patients.ConclusionsSegmental MRI Pituitary and Hypothalamus volumes post Fontan are increased and are related to the time since Fontan procedure. These findings are consistent with venous congestion of the anterior hypothalamic-pituitary portal venous system and may explain the high frequency of endocrine dysfunction in this patient group.

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Section: Discussionmentioning

confidence: 74%

Section: Introductionmentioning

confidence: 99%

Segmental MRI Pituitary and Hypothalamus Volumes post Fontan: An analysis of the Australian and New Zealand Fontan Registry

Gee,

Yang,

Gentles

et al. 2024

Preprint

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“…Pregnancies achieved by ART have also been associated with a higher risk of complications including preeclampsia and placental abruption [45]. In patients with Fontan circulation who are susceptible to complications of large fluids shifts, use of modified hormone protocols and single-embryo transfers may be used to minimize risk of ovarian hyperstimulation syndrome [42,46]. Decisions regarding safety of ART can be made in conjunction with an experienced cardio-obstetrics team [42].…”

Section: Preconception Counsellingmentioning

confidence: 99%

“…Patients with Fontan circulation who suffer from amenorrhea or irregular menstruation may have difficulty achieving pregnancy and seek assisted reproductive technologies (ARTs) [42]. There are little data to guide the implementation of ART among patients with complex ACHD.…”

Section: Preconception Counsellingmentioning

confidence: 99%

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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“…This condition is classified as a highly complex heart disease [17] and is associated with a higher risk of infertility, although with the correct management it may be possible to carry pregnancy to term [45]. Appropriate counselling in a tertiary care centre should be provided to assist the patient in planning her pregnancy [24].…”

Section: Fontan-type Univentricular Circulationmentioning

confidence: 99%

Pregnancy in Patients with Moderate and Highly Complex Congenital Heart Disease

et al. 2023

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Although not completely devoid of risk, pregnancy can be managed in virtually all patients affected by even the most complex forms of congenital heart disease. It is not however advisable in patients with any form of pulmonary arterial hypertension. Pregnancy is even manageable in patients with univentricular heart converted to Fontan circulation. A personalised risk stratification should be performed, and patients affected by advanced NYHA functional class appropriately warned of the potential risks. In this setting, metabolomics might represent a novel tool for use in conducting personalised risk stratification. All pregnancies, particularly those at higher risk, should be managed in a tertiary care centre capable of providing the necessary assistance to both the mother and infant. With a few rare exceptions, vaginal delivery is to be preferred over caesarean section due to the lower degree of maternal and foetal complications. The desire for motherhood, at times extreme in women with congenital heart disease, may often be accomplished, thus providing a ray of hope in the lives of these patients.

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Reproductive Issues in Patients With the Fontan Operation

Cited by 12 publications

References 69 publications

Segmental MRI Pituitary and Hypothalamus Volumes post Fontan: An analysis of the Australian and New Zealand Fontan Registry

Segmental MRI Pituitary and Hypothalamus Volumes post Fontan: An analysis of the Australian and New Zealand Fontan Registry

Management of the Fontan patient during pregnancy

Pregnancy in Patients with Moderate and Highly Complex Congenital Heart Disease

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