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Review
. 2025 Jun 26;17(6):106295.
doi: 10.4330/wjc.v17.i6.106295.

Navigating women with congenital heart disease during pregnancy: Management strategies and future directions

Bibhuti B Das  1 , Varun Aggarwal  2 , Shriprasad R Deshpande  3
Affiliations
Review

Navigating women with congenital heart disease during pregnancy: Management strategies and future directions

Bibhuti B Das et al. World J Cardiol. .

Abstract

Women with adult congenital heart disease (CHD) face unique challenges during pregnancy, as gestational cardiovascular (CV) and hemodynamic changes can exacerbate underlying cardiac conditions. While these adaptations are well tolerated in women with structurally and functionally normal hearts, they pose significant risks for those with adult CHD (ACHD), whether repaired, palliated, or with residual defects. Maternal CHD is associated with an increased risk of adverse CV events, including stroke, heart failure, arrhythmias, and thromboembolic complications during pregnancy and the peripartum period. Effective management requires a multidisciplinary team, including cardiologists, perinatologists, anesthesiologists, and other skilled care providers. Risk stratification tools such as the modified World Health Organization classification, CARPREG II, and ZAHARA scores are useful for predicting maternal and fetal outcomes and guiding clinical decision-making. Preconception counseling plays a critical role in assessing individual risks, optimizing cardiac function, and educating patients about potential complications. Future research should prioritize innovative therapies, including targeted pharmacological agents and minimally invasive interventions, alongside improved screening methods to identify high-risk patients before symptomatic disease manifests. This review synthesizes current literature on managing pregnant women with ACHD, highlights gaps in clinical practice, and explores future directions to enhance care. Addressing these challenges is essential to improving maternal and fetal outcomes and ensuring comprehensive, patient-centered care throughout the reproductive journey.

Keywords: Congenital heart disease; Contraception; Maternal & fetal outcomes; Postpartum care; Pregnancy; Women with congenital heart disease.

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Conflict of interest statement

Conflict-of-interest statement: There is no conflict of interest associated with any of the senior author or other coauthors contributed their efforts in this manuscript.

Figures

Figure 1
Figure 1
Cardio-obstetric team. ACHD: Adult congenital heart disease; EP: Electrophysiology; HF: Heart failure; L-D: Labor and delivery.
Figure 2
Figure 2
Overall summary of management of adult congenital heart disease during pregnancy. CO: Cardiac output; ACHD: Adult congenital heart disease; CV: Cardiovascular; SVR: systemic vascular resistance.
Figure 3
Figure 3
Modified World Health Organization classification for maternal risk associated with adult congenital heart disease. mWHO: Modified World Health Organization; PLE: Protein-losing enteropathy; CHD: Congenital heart disease; LV: Left ventricle; LVEF: Left ventricular ejection fraction; AS: Aortic stenosis; MS: Mitral stenosis; HCM: Hypertrophic cardiomyopathy; PS: Pulmonary stenosis; VSD: Ventricular septal defect; PDA: Patent ductus arteriosus; MVP: Mitral valve prolapse; ASD: Atrial septal defect; PAC: Premature atrial contraction; PVC: Premature ventricular contraction.
Figure 4
Figure 4
Risk stratification for women with ACHD during pregnancy. A: The risk of cardiac events per modified World Health Organization (mWHO) class; B: Composite risk comparison for cardiac events in women with adult congenital heart disease during pregnancy: mWHO class, CARPEG II, and ZAHARA scores. mWHO: Modified World Health Organization.
Figure 5
Figure 5
Adult congenital heart disease in women during pregnancy. A: Aortic stenosis, mitral stenosis, coarctation of the aorta; B: Unrepaired tetralogy of Fallot; C: Uncomplicated Ebstein anomaly; D: Extra-cardiac Fontan; E: Congenitally corrected transpositions of great arteries; F: Transposition of great arteries after Senning/mustard; G: Transposition of great arteries after arterial switch operation; H: Left to right shunt lesions (atrial septal defect, ventricular septal defect, and patent ductus arteriosus). ASD: Atrial septal defect; VSD: Ventricular septal defect; PDA: Patent ductus arteriosus; AO: Aorta; PA: Pulmonary artery; RV: Right ventricle; RA: Right atrium; LV: Left ventricle; LA: Left atrium; SVC: Superior venacava; IVC: Inferior venacava; TGA: Transposition of the great arteries arteries.
Figure 6
Figure 6
Postpartum care for women with adult congenital heart disease. CVD: Cardiovascular disease; IUGR: Intra-uterine growth retardation.
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