Pregnancy and Reproductive Health in Adult Congenital Heart Disease

Pregnancy and Reproductive Health in Adult Congenital Heart Disease

Evan F. Shalen

Abigail D. Khan


Innovations in the treatment of congenital heart disease (CHD) have greatly improved survival, leading to a dramatic increase in the size of the population of adults with CHD (ACHD).1 CHD is now the most prevalent type of heart disease in pregnant women worldwide.2 Reproductive health issues are of paramount importance as the CHD population ages into adulthood, and counseling about contraception and pregnancy is the cornerstone of ACHD care.3

ACHD comprises a diverse group of disorders with a wide range of expected clinical outcomes. The diversity of the underlying conditions and the changing nature of the surgical approach to ACHD have resulted in significant heterogeneity in pregnancy risk. Similarly, the risks associated with contraception and assisted reproductive strategies vary, presenting a challenge to those delivering care to this population. In this chapter, we provide an overview of ACHD pregnancy risk and address reproductive-related care for both men and women with ACHD.


An understanding of the hemodynamic changes of pregnancy is critical to the management of pregnant ACHD patients. The major cardiovascular changes associated with normal pregnancy are as follows:

  • An increase in the circulating plasma volume that begins by 6 weeks of gestation and peaks at 50% of baseline by 32 weeks.

  • A decrease in systemic vascular resistance and blood pressure.

  • An approximately 20% increase in heart rate.

  • Ventricular remodeling, leading to increased left ventricular (LV) end-diastolic volume with preserved LV ejection fraction (LVEF) and increased stroke volume, resulting in a 30% to 50% increase in cardiac output.

  • In active labor, 300 to 500 mL of blood is returned to the central circulation with each contraction, and cardiac output increases by 50%.

  • The decreased preload associated with the performance of the Valsalva maneuver during pushing results in a decrease in cardiac output in some patients.


Most cases of CHD are known prior to pregnancy. In rare cases, the physiologic changes of pregnancy and/or the increased intensity of medical care yields a new diagnosis of CHD. The clinical presentation is variable; however, women can present with murmurs, arrhythmias, or dyspnea and peripheral edema.


Overview of Risk Stratification of Pregnant Women With Congenital Heart Disease

There are three commonly used risk scoring systems for pregnant women with CHD, each of which has unique strengths and limitations, and the information that they provide should be viewed as complementary to information gleaned from smaller, lesion-specific cohorts.

Cardiac Disease in Pregnancy Study II (CARPREG II) Risk Score

The Cardiac Disease in Pregnancy (CARPREG II) score was developed in a cohort of 1938 women with various types of heart disease7 and identified 10 predictors of maternal cardiac events (Table 104.1). This score is easy to calculate, applies to women with and without CHD, and is appealing to clinicians and patients because it yields a user-friendly percentage risk. How the CARPREG II score performs in real-world cohorts has yet to be determined.

ZAHARA (Zwangerschap bij vrouwen met een Aangeboren HARtAfwijking-II [translated as “Pregnancy in Women with CHD II Risk Index”]) Risk Score

The ZAHARA score was developed in a large retrospective European cohort of women with CHD and identified eight factors associated with maternal cardiac complications (Table 104.1).8 Like CARPREG, ZAHARA calculates a percentage predicted risk but is less commonly used in the United States. While some risk markers are common to both CARPREG and ZAHARA, there are important differences, likely related to study methodology and the characteristics of the study populations.

World Health Organization Classification

The World Health Organization (WHO) classification is a qualitative scoring system that incorporates anatomic and physiologic features (Table 104.2).9 The WHO score performs well as a predictor of maternal, obstetric, and fetal events and has a higher discriminative ability than the CARPREG or ZAHARA scores.2,10 The WHO classification is the tool endorsed by major society guidelines.9,11

Risk Stratification by Defect Complexity

Simple Defects

Simple defects include small and uncomplicated atrial and ventricular septal defects (ASD, VSD); patent ductus arteriosus; mild pulmonic stenosis; and repaired ASD, VSD, and anomalous pulmonary venous return. These defects are associated with a minimally increased risk in pregnancy (Table 104.2).
Paradoxical embolus is a concern for those with persistent intracardiac shunts, and avoidance of venous stasis with compression stockings and early ambulation after delivery is recommended.

Moderate Complexity Congenital Heart Disease

Preconception counseling is strongly recommended for patients with moderate complexity CHD, and pregnant patients need to be followed by a cardiologist with CHD experience and a high-risk obstetrician. Preconception imaging is useful for risk stratification and hemodynamic optimization, whereas those at elevated risk of arrhythmias should have ambulatory rhythm monitoring. In some cases, exercise testing is helpful to predict cardiac events.6

Vaginal delivery is preferred unless there is an obstetric indication for cesarean section. Rare exceptions include those with decompensated heart failure or significantly impaired LV function, severe aortic enlargement, and/or ongoing poorly tolerated arrhythmias. A multidisciplinary delivery plan, including a discussion of the location and mode of delivery and the need for cardiac monitoring, is recommended.

Moderate and Large Unrepaired Atrial Septal Defects. Most moderate and large unrepaired ASD are WHO Class II (Table 104.2), although pregnancy is contraindicated if there is severe pulmonary arterial hypertension (WHO Class IV). Preconception cardiac MRI can be helpful to accurately size the right ventricle (RV), define the shunt fraction, and determine whether there is partial anomalous pulmonary venous return. Hemodynamically significant ASDs should be closed prior to pregnancy.3

Unrepaired ASD is associated with an increased risk of arterial thromboembolism, and strategies to reduce the risk of venous stasis are recommended. Women with unrepaired ASD can also develop atrial arrhythmias. Whereas those with large shunts and associated RV enlargement and/or dysfunction are theoretically at higher risk of volume overload, clinical heart failure is rare.12

Tetralogy of Fallot. Most women with repaired Tetralogy of Fallot (TOF) tolerate pregnancy well, with a reported maternal cardiac event rate of 7% or less.13,14 Heart failure and arrhythmias occur more frequently in women with RV dysfunction and/or moderate to severe pulmonary regurgitation.15 Overall complication rates are low, even in those with significant pulmonary stenosis or regurgitation.16,17

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May 8, 2022 | Posted by in CARDIOLOGY | Comments Off on Pregnancy and Reproductive Health in Adult Congenital Heart Disease
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