Abstract

Background: Conotruncal anomalies (CTA) are associated with ongoing dilation of the aortic root, as well as increased aortic stiffness, which may relate to intrinsic properties of the aorta. Pregnancy hormones lead to hemodynamic changes and remodeling of the tunica media, resulting in the opposite effect, i.e., increasing distensibility. These changes normalize post-pregnancy in healthy women but have not been fully investigated in CTA patients.Methods: We examined aortic distensibility and ventriculo-arterial coupling before and after pregnancy using cardiovascular magnetic resonance (CMR)-derived wave intensity analysis (WIA). Pre- and post-pregnancy CMR data were retrospectively analyzed. Aortic diameters were measured before, during, and after pregnancy by cardiac ultrasound and before and after pregnancy by CMR. Phase contrast MR flow sequences were used for calculating wave speed (c) and intensity (WI). A matched analysis was performed comparing results before and after pregnancy.Results: Thirteen women (n = 5, transposition of the great arteries; n = 6, tetralogy of Fallot; n = 1, double outlet right ventricle, n = 1, truncus arteriosus) had 19 pregnancies. Median time between delivery and second CMR was 2.3 years (range: 1–6 years). The aortic diameter increased significantly after pregnancy in nine (n = 9) patients by a median of 4 ± 2.3 mm (range: 2–7.0 mm, p = 0.01). There was no difference in c pre-/post-pregnancy (p = 0.73), suggesting that increased compliance, typically observed during pregnancy, does not persist long term. A significant inverse relationship was observed between c and heart rate (HR) after pregnancy (p = 0.01, r = 0.73). There was no significant difference in cardiac output, aortic/pulmonary regurgitation, or WI peaks pre-/post-pregnancy.Conclusions: WIA is feasible in this population and could provide physiological insights in larger cohorts. Aortic distensibility and wave intensity did not change before and after pregnancy in CTA patients, despite an increase in diameter, suggesting that pregnancy did not adversely affect coupling in the long-term.

Highlights

  • Conotruncal cardiac anomalies (CTA) include a variety of congenital heart defects, such as tetralogy of Fallot (ToF), truncus arteriosus (TA), double outlet right ventricle (DORV), and transposition of the great arteries (TGA)

  • There are a few hypotheses for why aorta dilation (AD) in Conotruncal anomalies (CTA) occurs principally

  • Recent evidence demonstrates that early ToF repair does not normalize aortic compliance and that abnormal ascending aortic flow patterns based on 4D flow cardiac magnetic resonance (CMR) are prevalent and might affect long-term cardiac performance [7]

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Summary

Introduction

Conotruncal cardiac anomalies (CTA) include a variety of congenital heart defects, such as tetralogy of Fallot (ToF), truncus arteriosus (TA), double outlet right ventricle (DORV), and transposition of the great arteries (TGA). These defects represent 5–10% of congenital heart disease [1] and generally lead to severe cyanosis, necessitating repair in the newborn period or early in infancy. The first is that AD occurs due to hemodynamic stress on the aorta from an early right-left shunt. Pregnancy hormones lead to hemodynamic changes and remodeling of the tunica media, resulting in the opposite effect, i.e., increasing distensibility These changes normalize post-pregnancy in healthy women but have not been fully investigated in CTA patients

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