The treatment is the same as in the nonpregnant patient. Complications include arrhythmia, heart failure, cardiogenic shock and death of both mother and baby.
Due to improved outcomes for patients with congenital heart disease (CHD), there is an increasing number of women with congenital heart disease who reach childbearing age [9,10]. In most cases, the woman is well known to adult congenital heart disease services where individualized prepregnancy counseling should take place. Individual risk is dictated by the nature and complexity of the defect, but in general those with more complex disease have a higher risk of obstetric complications. Maternal risk is strongly influenced by cardiac output and functional class prior to pregnancy, and during pregnancy women with CHD are at risk of arrhythmia. The presence of cyanosis is also relevant to offspring outcome: if maternal oxygen saturation is <85% the chance of a life birth is only 12% [11]. Prematurity and low birth weight are frequent complications.
Cardiomyopathy
Approximately a fifth of cardiovascular deaths in pregnancy are attributable to cardiomyopathy [1]. Hypertrophic cardiomyopathy (HCM) is often well tolerated with the appropriate specialist care and should not represent a contraindication to pregnancy. Preterm birth, however, occurs in a quarter of patients with HCM [12]. In dilating forms of cardiomyopathy (DCM), pregnancy can result in significant deterioration of LV function, and prepregnancy functional class III or IV and LV ejection fraction <40% are predictors of adverse outcome [13]. Prior to pregnancy, these women need careful counseling regarding the potentially irreversible deterioration of LV function and the risk of maternal and fetal death. All women with cardiomyopathy are at risk of arrhythmia, which may sometimes be poorly tolerated (e.g. atrial fibrillation in patients with HCM, or ventricular tachyarrhythmia).
Valve disease
Preexisting valve disease can decompensate in pregnancy due to the need to increase cardiac output and intravascular volume. Decompensated valve disease in pregnancy presents in the same way as in the nonpregnant patient with symptoms of heart failure and pulmonary oedema. Stenotic valve disease is generally less well tolerated than regurgitant valve disease. In some parts of the world the prevalence of rheumatic valve disease remains high and often undiagnosed. Mitral stenosis in particular is frequently unmasked by pregnancy, and women with severe mitral stenosis should be discouraged from (or intervened upon prior to) pregnancy. Mitral valvotomy may bridge time to more definitive valve replacement once family planning is complete. Women with severe symptomatic aortic stenosis should be counseled against pregnancy while those without symptoms, good LV function and normal exercise tolerance may tolerate pregnancy under close surveillance by a specialist team [14]. There is an increased risk for mother and baby, namely intrauterine growth restriction, preterm birth and low birth weight. Fetal death occurs in <5% mothers with severe stenotic valve disease [15]. Women with mechanical valves on anticoagulation are at high risk of complications. A 2017 study from the UK suggests that only 28% of pregnancies resulted in favorable outcome for mother and baby [16], and ROPAC reports event free pregnancy with live birth in 79% in women with bioprosthetic valves, but only 58% in women with mechanical valve prostheses [17]. For these women there is a high risk of both valve thrombosis but also bleeding related to the need for full anticoagulation throughout pregnancy.
Key points
Challenge: A patient with heart disease presenting for ART.
Background:
Ischemic heart disease:
A high index of suspicion is needed to diagnose, as women may present with atypical symptoms.
The treatment of pregnant or postpartum women with ACS is the same as for the nonpregnant patient.
Congenital heart disease:
Maternal risk is strongly influenced by cardiac output and functional class prior to pregnancy
During pregnancy women with CHD are at risk of arrhythmia.
Prematurity and low birth weight are frequent complications.
Cardiomyopathy:
Hypertrophic cardiomyopathy (HCM) is often well tolerated and should not represent a contraindication to pregnancy.
Preterm birth occurs in a quarter of patients with HCM.
In dilating forms of cardiomyopathy (DCM), pregnancy can result in significant deterioration of LV function.
Women with cardiomyopathy are at risk of arrhythmia.
Valve disease:
Stenotic valve disease is generally less well tolerated in pregnancy than regurgitant valve disease.
For the majority of patients with prosthetic valves pregnancy is safe provided appropriate anticoagulation is continued.
Substitution of warfarin with subcutaneous LMWH in combination with oral aspirin for the period of organogenesis and/or throughout the pregnancy, with monitoring of anti‐Xa levels is a safe option.
Management options:
Aim for single embryo transfer (to reduce the risk of multiple pregnancy and OHSS).
Reduce risk of OHSS (gentle COS, GnRH antagonist cycle with GnRH agonist trigger).
Prepregnancy assessment by an obstetric physician or a maternal medicine specialist and a cardiologist is mandatory.
A thorough discussion of maternal and fetal risks must take place.
Adjustment of medications as appropriate and referral to a multidisciplinary team as soon as pregnancy is confirmed.
If on LMWH, stop 24 hours before oocyte retrieval, and consider restarting on the evening of oocyte retrieval.
For women with mechanical heart valves, antibiotic prophylaxis for endocarditis prevention is not routinely indicated at oocyte retrieval.
Answers to questions patients ask
1 Q1 Is the pregnancy likely to make my heart disease worse? A1. Every woman with heart disease is different and should undergo preconception counseling by a cardiologist or obstetric physician trained to look after pregnant women with heart disease. Patients who have no or only minimal symptoms tend to do well during pregnancy, and there are very few women who need to be advised against pregnancy. Some women may require more frequent checks, and some may need to be delivered earlier than at term – the specialist team looking after you will advise if this is necessary. Formal assessment of cardiac reserve with exercise stress echo can be used before assisted reproduction to better inform prepregnancy counseling.
2 Q2 Will the medication I have been prescribed harm my baby? A2. Any medication prescribed by the specialist team looking after you is given to make the pregnancy safer for you and your baby. If you are prescribed a medication, which could potentially harm your baby, your specialist should alert you to this and discuss alternatives if there are any. Cardio‐selective betablockers
