Available online 25 November 2023

Severe neonatal Ebstein’s anomaly and tricuspid valve dysplasia (EA/TVD) are associated with high perinatal morbidity and mortality. We have recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. With this, we sought to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion/ Starnes on LV performance in neonatal EA/TVD.

We identified neonates with EA/TVD encountered from 2004-2018 in three institutions. Pre and postoperative LV function was assessed using 2D, Doppler-derived, deformation (6-segmental Vector Velocity Imaging), and two mechanical dyssynchrony measures (standard deviation of time-to-peak, T2PSD and global dyssynchrony index, DI), and values were compared using paired t-test analysis or Wilcoxon rank sum test.

Pre-intervention, PDA (n=18) and Starnes (n=6) group LV function was impaired and similar between groups. Post PDA closure, LV performance did not change. Post-Starnes, however, LV function including synchrony improved significantly: fractional area change Pre 44.6±4.6% vs. Post 57.9±7.9% p=0.003; global circumferential strain Pre -18.2±4.97 vs. Post -32.5±5.52, p=0.01; cardiac index Pre 1.9±0.3 vs Post 3.9±1.5 L/min/m2, p=0.05; and circumferential strain dyssynchrony (DI Pre 0.19±0.09 vs. Post 0.04±0.02; p=0.009 and T2PSD: Pre 59.8±18.5 vs. Post 29.9±8.22, p=0.02).

The Starnes procedure results in early improvements in LV dysfunction and dyssynchrony, not observed after PDA closure in neonatal severe EA/TVD, which may benefit the critically unwell neonate.

2023 Published by Elsevier Inc. on behalf of the American Society of Echocardiography.