Preconception Issues And Care

Ideally, all women should be able to make an informed choice about pregnancy based on both maternal and fetal risks, long term prognosis and alternative options such as contraception, termination of pregnancy, adoption, surrogacy and IVF. Encourage realistic expectations, and advocate active preparation for pregnancy. This includes:

• Ensuring easy access to multi-disciplinary care

• Genetic counselling

• Pre-pregnancy surgery or intervention: e.g. change of valve type; consider risk of surgery versus reduction in pregnancy risk; catheter interventions; ablation of arrhythmia; and optimisation of cardiac function

• Optimal timing of pregnancy (pregnancy at a younger age is lower risk for some complex conditions)

• Avoidance of teratogens (medication may need to be changed prior to pregnancy, e.g. anticoagulants, ACEI, anti-arrhythmics) (see Cardiac Drugs in Appendix 4.3)

• Prompt initiation of iron supplementation

• General measures: smoking cessation and folic acid supplementation

Pregnancy Issues

Each woman will need management according to individual anatomical and functional status.

Close liaison between cardiologists and obstetricians is essential.

• Admission for bed-rest +/- oxygen therapy may be required

• Anaemia should be avoided

• Fetal echocardiography is required as well as regular fetal wellbeing and growth monitoring

• Appropriate timing for delivery is crucial to balance maternal and neonatal morbidity and mortality

• A clear plan of management for labour and delivery should be established in advance, clearly documented and widely available

Medical Management and Care

• Iron deficiency - monitor red cell indices plus Hb, because cyanotic patients can be functionally anaemic even with a 'normal' Hb

• Warfarin may not be essential especially for Fontan patients; change to aspirin, or low-molecular-weight heparin

• Stop ACE inhibitors if not done pre-conceptually

• Regular outpatient review, echocardiography +/- Holter monitoring

• Beta-blockers for arrhythmia +/- pacemaker if indicated

• Diuretics for heart failure/breathlessness5

• Oxygen therapy may help severely cyanosed patients, especially if they have a reduced respiratory capacity

Midwifery Management and Care

• Early booking, with thorough history taking, and immediate referral to a maternal medicine clinic, then liaison with tertiary cardiologist

• Baseline observations, including oxygen saturation

• Maintain Hb levels as above, with additional dietary advice5

• Advise on antibiotic treatment for any dental or other procedures

• Teach the mother to keep a fetal movement chart after 24 weeks

• Prepare parents for potential pre-term/IUGR baby or fetal loss

Labour Issues

Labour requires careful monitoring of both mother and fetus. Pre-load and blood pressure should be monitored carefully and blood loss minimised. Antibiotic prophylaxis should be given during labour and delivery.

In general, vaginal delivery with low dose epidural is the mode of choice. Forceps or ventouse delivery may be used to shorten maternal expulsive effort in the second stage. Cardiac indications for caesarean section include aortic dilatation >40 mm, warfarin treatment and important systemic ventricular dysfunction.

Bolus doses of Syntocinon should be avoided in the third stage, as they can cause severe hypotension. Low dose oxytocin infusions are safer. Ergometrine is best avoided in most cases as it can cause acute hypertension. Uterine compression sutures are the most effective treatment in the management of uterine atony at caesarean section. The safety of misoprostol is yet to be determined.

Medical Management and Care

• Prophylactic (even temporary) pacemaker is considered for some women as pushing is vagotonic

• If normal birth is anticipated consider shortening the second stage

• If there is evidence of aneurysm formation, or major ventricular dysfunction, caesarean section is preferable6

• Epidural use is recommended

• Avoid supine position, especially for Fontan patients, because caval compression restricts pulmonary blood flow

• Invasive arterial +/- venous pressure monitoring may be helpful even for vaginal delivery

• Cyanotic patients may require intravenous hydration, especially if very nauseated

• Prescribe antibiotic prophylaxis

Midwifery Management and Care

• Intensive care setting is likely5; experienced staff required

• Administer the antibiotics and any other prescribed drugs as above

• Keep the mother in left lateral position throughout the first stage7

• The midwife may well be required to conduct a normal delivery under close medical supervision, with an emphasis on a short second stage, which could require an episiotomy

• Alternatively the role might be to assist with an instrumental/ operative delivery, which may well be pre-term

• Otherwise the care is as in Section 4.1

Postpartum Issues

Following delivery, the return of the uterine blood flow into the systemic circulation results in an increase of cardiac output. Stroke volume, heart rate and cardiac output remain high for 24 hours post-delivery with rapid intravascular volume shifts in the first two weeks postpartum, thus the later stages of labour and early postpartum period are times of high risk for pulmonary oedema1.

• The neonate may require intensive care in addition to heart screening

• Contraceptive issues are similar to the previous section

• Most require progesterone-only methods

Medical Management and Care

• Close medical supervision is still required after delivery with monitoring for at least 48 hours

• Review medications prior to discharge: restart ACE inhibitors and warfarin; some beta-blockers are excreted less in breast milk

• Systemic ventricular dysfunction may continue or worsen post-partum so careful review pre-discharge is required

• Cardiac follow-up 4-6 weeks post-delivery for almost all patients

Midwifery Management and Care

• This mother is not for discharge until reviewed by the medical team

• Basic post-operative care is required if post-caesarean section

• Basic observations may need continuation for longer than usual with emphasis on oxygen saturation, blood pressure and fluid balance

• Advise and support specific to the needs of a mother with a baby on a neonatal unit if delivery was pre-term (see Chapter 1)

• Bereavement care if a stillbirth occurred

• The basic care is as in Section 4.1


Prevalence varies as to the individual condition

Risk for Childbearing

Moderate to High Risk

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  • Jan
    Why keep supine and up position forfontan?
    7 years ago

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