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Pubertal development and onset of menstruation

Onset may be later

Menstrual cycle concerns, contraception and fertility planning

Development of regular cycles – can be affected by body weight, stress, illness
Contraception – options available affected by menstrual pattern, clot risk
Fertility – studies suggest lower ovarian reserve, higher need for assisted conception

Pregnancy, delivery and postpartum care

Prepregnancy cardiac assessment and team development
Monitoring of cardiac function, rhythm, fluid balance; fetal growth and placental function

Perimenopause and menopausal transitions

Earlier onset, heavy bleeding secondary to venous pressures
Therapeutic options limited by special considerations for Fontan circulation

Gynecologic Care Across the Life Span:

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Delayed onset of growth spurt, pubertal development in some studies

May be related to being born smaller for dates/premature
May also be related to decrease ovarian reserve
Investigations with hormone and genetic testing if more than 3 years behind peers

Menstrual cycle onset may be later than peers

Heavier flow common – often treated by GPs, peds gynecology

Special Considerations for Puberty:

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Cycle pattern

Irregularity of periods more common

Ovulation cadence less regular
Longer cycles tend to be heavier

Intermenstrual spotting

Heavier menstrual bleeding

More common in higher hemoglobin levels, worse ventricular function
Need to rule out other common causes

Fibroids, polyps, uterine anomalies

Management options for management depend upon

Underlying thrombosis risk/use of anticoagulation
Desire for future fertility

Menstrual Cycles:

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Condoms will not address menstrual concerns but only one with STI protection

Menstrual flow may limit utility of copper IUD

Often increase blood loss with cycles

Estrogen containing contraceptives carry a risk of clotting

Superior control of heavy bleeding for some patients on anticoagulation for short term

Progesterone only contraceptive generally an excellent option

May be oral, implantable, IUD, injection
Good control of heavy bleeding but may have increase in spotting
May be used as emergency contraception
Common side effects include breast tenderness, mood changes

Contraceptive Concerns:

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Spontaneous conception is possible if having cycles with ovulation

Delayed conception common due to multiple reasons

Impaired ovulation /ovarian reserve
Impaired uterine blood flow/oxygenation

Fertility assistance options carry own risks and benefits

Ovulation assistance – may cause higher estrogen and clot risk
IVF increases the risk of blood clotting / bleeding around hormone stimulation

Fluid overload risks can be decreased by using single frozen embryo

Fertility Planning and Concerns:

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Pregnancy ought to be a planned event in individuals with a Fontan circulation.
Pre pregnancy appointment in cardiac obstetrics (COB) clinic:

Status and history of any Fontan complications (Heart Failure, arrhythmias, blood clots)
Physical assessment (weight, cyanosis, oxygen saturations)
Heart Rhythm (ECG)
Echocardiogram (heart function assessment)
Cardiopulmonary exercise test (exercise capacity)
Cardiac MRI (to look for any Fontan circulation obstructions)
Assessment of liver and kidney function

Pre pregnancy assessment:

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Fertility Challenges in women with Fontan circulation

Increase in rate of miscarriage to 40 – 70% (compared to ~ 15% in general population). Potential causes:

Lower oxygen levels in the blood decreases the ability of the fetus to survive
Fontan complications such as abnormal valve function or heart failure
Pregnancy terminations reported to occur ~7 – 10% -- may also contribute to low live birth rates

Increased rates of bleeding in first and second trimester

May settle spontaneously or be ongoing

Wichert-Schmitt, D’Souza, Silversiders, 2022

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Wichert-Schmitt, D’Souza, Silversiders, 2022

CJC July 2022 Figure 2. Pregnancy risks in patients with the Fontan operation.

Maternal cardiac complications:

Heart failure

Diastolic or systolic dysfunction can occur following Fontan – predisposing patients to HF
heart failure was reported in 3.9% of pregnancies (predominantly in the third trimester and postpartum)
Symptoms of heart failure should trigger the search for an underlying cause (arrhythmias, obstruction of the Fontan pathway, eg, due to pulmonary embolism, or pregnancy-associated complications (hypertension, preeclampsia, anemia, and bleeding).
Treatment of HF should take into account the underlying cause, as conventional heart failure medication may not be helpful in the Fontan population, particularly in the absence of systolic ventricular dysfunction.
Diuretics should be administered to women with volume overload or pulmonary edema and used cautiously, as the Fontan circulation depends on adequate preload, and dehydration can have deleterious effects on maternal and fetal health.

Most frequent complication = risk of arrhythmias (most frequently SVT)

8.4% of Fontan pregnancies complicated by arrhythmias (mainly in 3^rd trimester) – restoration of sinus rhythm

Thromboembolic events

hypercoagulable state of pregnancy in conjunction with the Fontan circulation elevates the risk of thromboembolism during pregnancy. Thromboembolism can occur in the pulmonary as well as the systemic circulation and is associated with high morbidity and mortality.
review of the literature, thromboembolic complications were found to occur in 1.7% of pregnancies. ⁸ Outside of pregnancy, the risk of thromboembolism is higher in patients with the older atrio-pulmonary Fontan operations, compared to the risk in those with a lateral tunnel or an extracardiac conduit, and this difference is likely present during pregnancy as well

Obstetrics

Bleeding !%%

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Wichert-Schmitt, D’Souza, Silversiders, 2022

CJC July 2022 - Adverse pregnancy outcomes in patients with the Fontan operation in achieved and maintained pregnancies. Adapted from Garcia Ropero et al.⁸

Maternal obstetric complications:

Bleeding - In the Fontan population, obstetric bleeding is increased, and postpartum hemorrhage is reported in 14% of pregnancies.

bleeding risk associated with the Fontan circulation and the use of antithrombotic medication contribute to the high rates of obstetric bleeding
thrombotic and bleeding risks🡪 individualized antithrombotic strategy should be decided upon in conjunction with the patient.

Fetal and neonatal complications

limited ability of the maternal cardiovascular system to increase uteroplacental blood flow, high venous pressure, hypoxemia, and maternal cardiac medication can all negatively impact the developing fetus.
preterm deliveries are reported in 59% of live births, with average gestational ages between 32 and 34 weeks.
Prematurity can be secondary to premature rupture of membranes and antepartum vaginal bleeding, but it also may be iatrogenic, with physicians deciding to perform preterm deliveries in the setting of fetal intrauterine growth restriction or maternal cardiac deterioration.
In one systematic review of 255 pregnancies (115 live births) in patients with a Fontan circulation, 20% of live births were reported to be small for gestational age ⁸ ; mean birthweights varied between 1500 and 2100 grams, depending on the study. ²⁹ ^, ³⁰ ^, ³⁶ ^, ³⁷ ^, ⁵⁶
Rates of stillbirth and neonatal deaths are increased, with neonatal deaths as high as 5%.
Transmission of congenital heart disease occurs in 3%-5% of offspring. However, transmission of heart disease depends on the underlying cardiac lesion and is higher in syndromic patients and those with atrioventricular septal defects or hypoplastic left heart syndrome. ⁴⁰ ^, ⁶¹ A fetal echocardiogram, often performed between 18 and 22 weeks gestation, is recommended to screen for congenital heart defects. ²² ^, ⁴⁰ These high rates of fetal complications highlight the need for close fetal monitoring throughout pregnancy, by an experienced obstetric team.

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Pregnancy Management

Pregnancy care should be provided by an experienced cardio-obstetric team
Monitoring plan should be established early in pregnancy
A labour and delivery care plan should be prepared by the multidisciplinary cardio-obstetric team and circulated well before delivery
Availability of experienced cardio obstetrics team will determine delivery location

Davis, M.B. et al., JACC 2021

care should be provided by an experienced cardio-obstetric team.
no adequate preconception counselling, counselling should occur in the early antenatal period.
surveillance plan for the pregnancy should be established, and the frequency of follow up should be modified as needed.

A labour and delivery care plan should be prepared by the multidisciplinary cardio-obstetric team and should be circulated well before delivery. This plan should outline:

the mode and timing of birth,
need for labour induction,
timing and nature of labour analgesia and anaesthesia
provide details for management of labour and childbirth, including maternal and fetal surveillance, use of uterotonic agents, need for infective endocarditis prophylaxis, and the timing and dose for resumption of postpartum anticoagulation.
The plan should also anticipate potential cardiac, obstetric, and fetal and/or neonatal complications and the resources needed to address them should they occur. ²¹ ^, ⁴⁰

A planned vaginal birth is recommended in most stable cardiac patients, including those with high-risk cardiac lesions, ⁶² ^, ⁶³ yet only one-third of Fontan patients have a vaginal birth. ⁸ ^, ²⁹ A vaginal birth does have advantages. For instance, the amount of blood loss during vaginal birth under regional analgesia is usually less that that with a Cesarean delivery. Obstetric and anaesthetic procedures that result in excessive blood loss or decreases in blood pressure should be avoided when possible, as these are poorly tolerated by the preload-dependent Fontan circulation. ⁵⁹ ^, ⁶⁴ Monitoring of blood pressure and heart rate during labour and childbirth is important, although invasive hemodynamic monitoring is rarely necessary. If the patient has an increased risk of arrhythmia, telemetry can be used from the onset of labour until the early postpartum period. ⁴¹

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The Fourth Trimester: Postpartum Concerns

Most patients will be on antecoagulation postpartum even if not during pregnancy

Vaginal bleeding may be prolonged or heavier

Breastfeeding should be unaffected

Milk production can be reduced if diuresis is needed for fluid overload
Some medications for heart failure may be not recommended for breastfeeding

Progesterone only contraception safe for both Fontan and for breastfeeding

Wichert-Schmitt, D’Souza, Silversiders, 2022

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Menopause is diagnosed after 12 months of absent menstruation

Any bleeding after this must be investigated (biopsy, ultrasound)
Average onset is 51 but is earlier in Fontan circulation

Symptoms may arise up to 5-10 years prior to cessation of menstruation

Menstrual irregularity increases
Hot flushing/sleep disturbance/concentration
Pelvic floor changes – bladder function/sexual function/vaginal dryness
Also reported – joint pain, weight gain, mood changes

Hormone therapy (primarily estrogen) is first line for symptoms but does carry risk

Low dose vaginal estrogen is safe and can help pelvic floor issues
Low dose transdermal estrogen has less clot risk that oral tablets

Would need to balance with cardiology the risks

Progesterone needed for prevention of uterine cancer unless hysterectomy performed

Menopause and Perimenopause

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Irregular and heavy bleeding is common in the perimenopause

Investigations include: blood testing, ultrasound, Pap testing, endometrial biopsy

Options for management depend upon

Age at onset and fertility desires
Presence of other conditions – endometriosis, fibroids, breast pathology

In general hormonal and surgical options are the mainstay

Hormonal options are as per contraception (Mirena, oral progesterone) with possible addition of estrogen for perimenopausal symptoms

Troublesome Bleeding

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Surgical Options for Troublesome Bleeding

Hysteroscopy

Involves looking inside the uterus and possible removing lesions causing bleeding
Day care procedure and carries little anaesthetic / cardiac risk
Pregnancy possible following completion

Endometrial ablation

The lining of the uterus is burned to prevent periods
Has a longer effective period in 40s, does not allow pregancny

Uterine artery embolization

Blocks blood flow to the uterus and decreases blood flow
Radiologic procedure with minimal bleeding risk
Not compatible with pregnancy, not helpful in pelvic pain

Uterine fibroid treatment or hysterectomy

Requires general anaesthetic which carries some cardiac risk
Laparoscopy may not be well tolerated for longer procedures due to pressures in the abdomen
Allows treatment of other problems (endometriosis, pelvic pain, scar tissue)

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PHC Heart CentrePresentation Template

14 May 2025

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Take Home Points

Ovarian function and menstrual cycles are affected by the Fontan circulation
Pregnancy should be planned and followed by an expert team to get the best outcomes
Contraception is important for timing of pregnancies and management of heavy bleeding
Menopause may come earlier, and treatment options are altered by cardiovascular risk

Wichert-Schmitt, D’Souza, Silversiders, 2022