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CardioNerds (Amit Goyal and Daniel Ambinder), ACHD series co-chair Dr. Agnes Koczo (UPMC), and ACHD FIT lead Dr. Katia Bravo (UCLA) join ACHD expert Dr. Carole Warnes (Professor of Medicine and founder of the Adult Congenital Heart Disease Clinic at Mayo Clinic), to discuss adult congenial heart disease and pregnancy. They cover preconception counseling in women with congenital heart disease, appropriate risk stratification to estimate maternal and neonatal morbidity using existing tools and an individualized care approach and preparation for a multidisciplinary delivery plan. Audio editing by CardioNerds Academy Intern, Dr. Leticia Helms.

The CardioNerds Adult Congenital Heart Disease (ACHD) series provides a comprehensive curriculum to dive deep into the labyrinthine world of congenital heart disease with the aim of empowering every CardioNerd to help improve the lives of people living with congenital heart disease. This series is multi-institutional collaborative project made possible by contributions of stellar fellow leads and expert faculty from several programs, led by series co-chairs, Dr. Josh Saef, Dr. Agnes Koczo, and Dr. Dan Clark.

The CardioNerds Adult Congenital Heart Disease Series is developed in collaboration with the Adult Congenital Heart Association, The CHiP Network, and Heart University. See more

Claim free CME for enjoying this episode! Disclosures: None

Pearls • Notes • References • Guest Profiles • Production Team

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Pearls

1. Women with congenital heart disease can safely carry a pregnancy if appropriate risk stratification and management is performed by an expert multidisciplinary cardio-obstetrics team.
2. There are a myriad of hemodynamic and vascular changes which impact congenital cardiac physiology and evolve throughout the different stages of pregnancy. Therefore, planning is key. An evaluation of heart rate response to exercise and optimization of potential factors that could worsen during pregnancy, such as arrhythmias, are recommended.
3. Maternal morbidity & mortality risk-stratification systems such as the modified WHO, CARPREG or ZAHARA criteria are helpful starting points. Ultimately, each patient requires individualization given the heterogeneity that exists among congenital heart defects.
4. Vaginal delivery is generally preferred over C-section from a cardiac standpoint. An assisted second stage of labor during the period of greatest hemodynamics changes should also be considered if vaginal delivery is pursued.

Show notes

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1. Why is preconception counseling important in women with congenital heart disease and what does it entail?

• Ensuring women with congenital heart disease are optimized prior to conception decreases maternal and neonatal complications. For this reason, preconception counseling is paramount for these patients.
• In addition, counseling leads to patients making more informed decisions about family planning.
• The integral parts of preconception counseling include: (1) discussing the current anatomical and physiological status, (2) discussing possible complications during pregnancy specific to the patient’s congenital heart defect (with genetic counseling referral if appropriate), (3) evaluating cardiac medications, taking into account benefits vs teratogenic potential, (4) discussing and planning for necessary pre-pregnancy tests or interventions (exercise stress testing is an important tool in evaluating how the patient will tolerate the stressors of pregnancy), (5) organizing antepartum care with high-risk obstetrics teams, (6) discussing location, timing and mode of delivery, (7) discussing alternative options to carrying a pregnancy in women who are at extreme risk (ie Fontan physiology, Eisenmenger syndrome) and (8) discussing postpartum contraception. 

2. How do we risk stratify women with congenital heart disease who are contemplating pregnancy?

An expert evaluation by an ACHD specialist is recommended using a thorough clinical and hemodynamic assessment centered around the individual patient and her values. Several risk prediction tools are available and should be used as a starting point. These include the modified World Health Organization (mWHO) classification, risk factors derived from CARPREG II (CARdiac disease in PREGnancy II), and ZAHARA (acronym based on Dutch translation for Pregnancy in congenital heart defects – Zwangerschap bij Aangeboren HARtAfwijkingen I) studies. 

• The mWHO classification groups congenital hearts conditions into class I to IV, with higher classes indicating higher predicted risk of cardiovascular maternal morbidity and mortality.
• The CARPREG II risk prediction index assigns points based on a number of different predictors including prior cardiac events or arrhythmias, baseline New York Heart Association Class III-IV or cyanosis, presence of a mechanical valve, ventricular dysfunction, high risk left-sided valve disease or left ventricular outflow tract obstruction, pulmonary hypertension, and coronary artery disease or high-risk aortopathies.
• The ZAHARA risk score also considers sub-pulmonary atrioventricular valve regurgitation and cardiac medications before pregnancy as additional risk factors as well.

3. What are the potential fetal/neonatal risks associated with pregnancy in congenital heart conditions?

Some of the risks include spontaneous abortion, premature birth, small for gestational age neonate, and congenital heart disease in the baby (which may be different than maternal CHD (see Table 1). Most of these are related to maternal heart failure; however other complications such as arrhythmias, endocarditis, thromboembolic events, and medication adverse effects predispose to neonatal complications. Specifically, women with uncorrected cyanotic heart disease and pulmonary hypertension with saturation levels ≤ 85% reach a live birth rate of only 12%.

Table 1. Recurrence rate for congenital heart lesions in affected mothers.

Lesion	Recurrence rate
Atrial septal defect	4-6%
Ventricular septal defect	6-10%
Atrioventricular septal defect	11.5-14%
Coarctation of the aorta	4-6.5%
Left ventricular outflow tract obstruction	8-18%
Right ventricular outflow tract obstruction	4-6.5%
Tetralogy of Fallot	2-2.5%
Transposition of the great arteries	2%
Congenitally corrected transposition of the great arteries	3-5%
Univentricular hearts	21%

What should a multidisciplinary delivery plan include?

Detailed delivery planning should be undertaken prior to expected delivery date to plan for obstetric anesthesia and analgesia, inpatient peripartum management (level of acuity, need for bubble filters in cases of shunts, avoidance of excessive volume load, infective endocarditis prophylaxis, mode of delivery), as well as postpartum care and discharge planning  including post-partum contraception.

Maternal and Neonatal Risk by Congenital Lesion

Anatomy	Maternal Risk	Fetal/ Neonatal risk	Points to consider
Right ventricular outflow tract obstruction or regurgitation	– Increased pregnancy-induced preload can promote RV dilatation and heart failure	– Preterm labor – Small for gestational age	Usually well tolerated during pregnancy
Left-sided obstructive lesions	– Heart failure in patients with severe symptomatic AS with volume expansion in pregnancy	– Small for gestational age	Balloon valvuloplasty in severe, symptomatic cases
Systemic right ventricle	– Heart failure – Atrial arrhythmias – Worsening systemic tricuspid regurgitation	– 25-50% prematurity – Small for gestational age	Systemic RV function may be impacted by repeat pregnancies
Coarctation of the aorta	– Hypertensive disorders of pregnancy – Heart failure	– Small for gestational age	– Hormonal changes may impact aortic vasculature into the postpartum period
Ebstein’s anomaly	– Cyanosis – Arrhythmias can be triggered by increased preload	– Related to maternal oxygen saturation	– Use bubble filters to avoid paradoxical emboli (high coexistence of ASD)
Fontan circulation	– Atrial arrhythmias can be triggered by increased preload – Thromboembolism due to hormonal changes – Discuss termination of pregnancy in failing Fontan	– 40-70% prematurity – Small for gestational age	Baseline oxygen saturation is the most important marker for fetal outcomes.
Eisenmenger syndrome   Also see Episode #124 – Pregnancy & Pulmonary Hypertension with Dr. Candice Silversides	– Mortality 20-40%. – Discuss termination of pregnancy	-Right ventricular failure from increased preload -Thromboembolism due to hormonal changes
Aortopathy   Also see Episode #126 – Pregnancy & Aortic Disorders with Dr. Nupoor Narula	– Hormonal changes impact aortic vasculature and can led to accelerated aortic enlargement – 10% risk aortic dissection risk (usually third trimester or postpartum) in Marfan syndrome with root diameter >40 mm, rapid dilation or previous dissection of the ascending aorta	– 15% premature delivery due to premature rupture of membranes. – In cases of dissection, fetal demise can occur.	Prophylactic aortic root replacement considered before pregnancy if diameter >40 mm, or smaller with rapid increase in dimensions, family history of dissection, or concomitant severe AI

References

1. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. Apr 2 2019;139(14):e698-e800. https://www.ahajournals.org/doi/10.1161/CIR.0000000000000603
2. European Society of G, Association for European Paediatric C, German Society for Gender M, et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). European heart journal. Dec 2011;32(24):3147-3197. https://academic.oup.com/eurheartj/article/39/34/3165/5078465
3. Pierpont ME, Brueckner M, Chung WK, et al. Genetic Basis for Congenital Heart Disease: Revisited: A Scientific Statement From the American Heart Association. Circulation. Nov 20 2018;138(21):e653-e711. https://www.ahajournals.org/doi/10.1161/CIR.0000000000000606
4. Elkayam U, Goland S, Pieper PG, Silversides CK. High-Risk Cardiac Disease in Pregnancy: Part II. Journal of the American College of Cardiology. Aug 2 2016;68(5):502-516. https://www.jacc.org/doi/full/10.1016/j.jacc.2016.05.048

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Guest Profiles

CardioNerds Adult Congenital Heart Disease Production Team

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