Impact of Fetal Somatic Growth on Pulmonary Valve Annulus Z-Scores During Gestation and Through Birth in Patients with Tetralogy of Fallot

Abstract

Previous studies have suggested reduced pulmonary valve annulus (PVA) growth and progression of pulmonary outflow obstruction in fetuses with tetralogy of Fallot (TOF). The goals of this study were to (1) investigate the trajectory of PVA growth in utero, and (2) compare two methods of z-score determination for fetal and postnatal PVA size by echocardiography in order to improve prenatal counseling for patients with TOF. Fetal echocardiograms (FE) at a single institution with a diagnosis of TOF between 8/2008 and 12/2015 were retrospectively reviewed. Patients included had at least 2 FEs and 1 immediate postnatal echocardiogram (TTE). Fetal and postnatal demographic, clinical, and echocardiographic data were collected. Fetal body surface area (BSA) was calculated by estimating fetal weight and height; z-scores were determined based on fetal gestational age (GA) and BSA for both FEs and TTEs. Fetal PVA z-scores by GA or BSA were then compared to postnatal PVA z-scores by BSA. Twenty-two patients with 44 FEs and 22 TTEs were included. GA at the first FE was 23 weeks ± 3.4 and 32 weeks ± 3.1 at the second FE. There was no difference in PVA z-scores (by BSA) between the first and second FE (p = 0.34), but a decrease in PVA z-scores (by BSA) between the second FE and TTE (− 1.6 ± 0.5 vs. − 2.0 ± 0.7; p = 0.01). Repeat comparison with fetal PVA z-scores indexed to GA revealed no difference in z-scores between the first and second FE, but an increase in PVA z-scores between the second FE (by GA) and TTE (by BSA) (− 4.1 ± 1.0 vs. − 2.0 ± 0.7; p < 0.0001). The rate of PVA growth between the two FEs (23 µm/day ± 9.8) and between the second FE and TTE (28 µm/day ± 42) remained comparable (p = 0.57); however, the rate of BSA increase was greater in later gestation (9 cm2/day ± 3 vs. 20 cm2/day ± 11; p = 0.001). In patients with TOF, the rate of PVA growth appears to remain consistent through gestation; however, somatic growth rate increases in late gestation. Fetal PVA z-scores indexed to GA are thus inaccurate in predicting postnatal PVA z-scores typically indexed to BSA. This observation should be considered during prenatal consultation and delivery planning.