Pregnancy in Patients with Moderate and Highly Complex Congenital Heart Disease
Abstract
:1. Introduction
2. Adaptation of the Cardiovascular System during Pregnancy
2.1. The ROPAC Registry (Registry of Pregnancy and Cardiac Disease)
2.2. Risk Stratification
2.3. Tetralogy of Fallot
2.4. Ebstein Anomaly
2.5. Systemic Right Ventricle
2.6. Pulmonary Hypertension
2.7. Fontan-Type Univentricular Circulation
2.8. Mechanical Valves
2.9. Using Cardiovascular Drugs during Pregnancy
2.10. Looking Ahead, the Outlook on Personalised Risk Stratification
3. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Pathophysiology | Specific Congenital Heart Disease | Pre-Pregnancy Management | During Pregnancy Management | Post-Pregnancy Management |
---|---|---|---|---|
1. CHD with increased pulmonary blood flow (septal defects without pulmonary obstruction and with left-to-right shunts) | Scimitar syndrome, interatrial septal defect, complete atrio-ventricular defect, ventricular septal defect, truncus arteriosus, aorto-pulmonary window, patent ductus arteriosus | Counselling (generally mWHO I, unless there is moderate mitral stenosis or post-surgical left ventricular impairment with ejection fraction 30–45% (mWHO III) or severe < 30% (mWHO IV). If truncal valve insufficiency is corrected with a mechanical valve, mWHO risk class is III) | 1–2 examinations/9 months for mWHO I; monthly or bimonthly examination for mWHO III; termination or monthly/bimonthly examination for mWHO IV | Discontinuation of heparin after 6 weeks from delivery in those with residual atrial septal defects. Six weeks post-delivery follow-up in mWHO III and IV |
2. CHD with decreased pulmonary flow (septal defects with pulmonary obstruction and with right-to-left shunt) | Pulmonary valve stenosis with atrial septal defect, pulmonary stenosis with ventricular septal defect (Tetralogy of Fallot), tricuspid atresia, Ebstein anomaly, single (double inlet) ventricle with pulmonary stenosis | Counselling (mWHO II–IV for Tetralogy of Fallot; mWHO II–III for Ebstein anomaly; mWHO III–IV for single ventricle corrected according to Fontan) | Examination every three months for mWHO II; monthly or bimonthly examination for mWHO III; termination or monthly/bimonthly examination for mWHO IV | Six weeks post-delivery follow-up in mWHO III and IV |
3. CHD with obstruction to blood progression and no septal defects (no shunt) | Pulmonary stenosis, aortic stenosis, coarctation of the aorta (adult type) | Counselling (mWHO II-III for severe pulmonary stenosis; mWHO III for asymptomatic severe aortic stenosis; mWHO IV for symptomatic severe aortic stenosis) | Examination every three months for mWHO II; monthly or bimonthly examination for mWHO III; termination or monthly/bimonthly examination for mWHO IV | Six weeks post-delivery follow-up in mWHO III and IV |
4. CHD so severe as to be incompatible with postnatal blood circulation | Ductus dependent CHD (pulmonary atresia, aortic and mitral severe stenosis/atresia, aortic arch obstruction), parallel and pulmonary circulations (complete transposition of the great vessels), anomalous connection/obstruction of the pulmonary veins (total anomalous pulmonary venous drainage, cor triatriatum sinister) | Counselling (mWHO II–IV for pulmonary atresia; mWHO IV for severe mitral stenosis; mWHO II–IV depending on the degree of aortic coarctation; mWHO I for transposition of the great vessels corrected with arterial switch; mWHO III–IV for transposition of the great vessels corrected with atrial switch | 1–2 examinations/9 months for mWHO I; monthly or bimonthly examination for mWHO III; termination or monthly/bimonthly examination for mWHO IV | Six weeks post-delivery follow-up in mWHO III and IV |
5. CHD silent until adult age | Bicuspid aortic valve, congenitally corrected transposition of the great vessels | Counselling (mWHO III for bicuspid aortic valve with moderate aortic dilatation or congenitally corrected transposition of the great vessels with good or mildly decreased right ventricular function; mWHO IV for bicuspid aortic valve with severe aortic dilatation or congenitally corrected transposition of the great vessels with moderately or severely decreased right ventricular function | Monthly or bimonthly examination for mWHO III; termination or monthly/bimonthly examination for mWHO IV | Six weeks post-delivery follow-up in mWHO III and IV |
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Panebianco, M.; Perrone, M.A.; Gagliardi, M.G.; Galletti, L.; Bassareo, P.P. Pregnancy in Patients with Moderate and Highly Complex Congenital Heart Disease. Healthcare 2023, 11, 1592. https://doi.org/10.3390/healthcare11111592
Panebianco M, Perrone MA, Gagliardi MG, Galletti L, Bassareo PP. Pregnancy in Patients with Moderate and Highly Complex Congenital Heart Disease. Healthcare. 2023; 11(11):1592. https://doi.org/10.3390/healthcare11111592
Chicago/Turabian StylePanebianco, Mario, Marco Alfonso Perrone, Maria Giulia Gagliardi, Lorenzo Galletti, and Pier Paolo Bassareo. 2023. "Pregnancy in Patients with Moderate and Highly Complex Congenital Heart Disease" Healthcare 11, no. 11: 1592. https://doi.org/10.3390/healthcare11111592
APA StylePanebianco, M., Perrone, M. A., Gagliardi, M. G., Galletti, L., & Bassareo, P. P. (2023). Pregnancy in Patients with Moderate and Highly Complex Congenital Heart Disease. Healthcare, 11(11), 1592. https://doi.org/10.3390/healthcare11111592