The perinatal transition is characterized by acute changes in cardiac loading. Compared with normal newborn combined cardiac output (CCO), single right ventricular (RV) output of neonates with hypoplastic left heart syndrome (HLHS) is markedly greater. The aim of this study was to examine the mechanisms of cardiac adaptation that facilitate this perinatal transition from late fetal to early neonatal life in HLHS.

Prospectively recruited pregnancies complicated by fetal HLHS (n = 35) and healthy control subjects (Ctrl; n = 17) underwent serial echocardiography in late gestation (38 ± 1 weeks) and 6, 24, and 48 hours after birth. Cardiac function was assessed using conventional, Doppler tissue, and speckle-tracking echocardiography.

Term fetuses with HLHS had RV output comparable with Ctrl CCO via higher stroke volume. Compared with both left ventricular and RV indices of Ctrl, they exhibited globular and dilated right ventricles with reduced relative wall thickness (0.40 ± 0.08 vs 0.49 ± 0.10, P < .01), increased Tei index′ (HLHS vs Ctrl left ventricle/Ctrl right ventricle: sphericity index, 0.9 ± 0.25 vs 0.5 ± 0.10/0.6 ± 0.11; RV area index, 28 ± 6 vs 15 ± 3/17 ± 5 cm²/m²; Tei index′, 0.65 ± 0.11 vs 0.43 ± 0.07/0.45 ± 0.09; P < .0001 for all). Neonates with HLHS generated elevated RV cardiac output compared with Ctrl CCO via higher heart rate and stroke volume, with further RV dilatation, increased longitudinal systolic strain at 48 hours (−17 ± 4% vs −14 ± 3%/ 14 ± 5%) with reduced circumferential and rotational myocardial deformation and altered diastolic function. Neonates with HLHS also demonstrated right atrial enlargement with increased longitudinal strain: 6 hours (33 ± 12% vs 26 ± 6%), 24 hours (37 ± 15% vs 26 ± 13%), and 48 hours (38 ± 11% vs 24 ± 13%) (P < .0001).

Term fetuses with HLHS exhibit altered RV geometry and RV systolic and diastolic functional parameters. After birth, further alterations in these cardiac parameters likely reflect adaptation to acutely altered RV loading from increasing cardiac output and pulmonary artery flow demands.

Geometric and functional cardiac alterations in term fetuses and neonates with HLHS. Speckle-tracking myocardial deformation curves showing systolic peaks of RV longitudinal strain (A) and apical rotation (B) in neonates with HLHS corresponding to the regional deformational parameters in six segments of the RV myocardial wall in the RV four-chamber view and apical short-axis view, respectively. The spider-web plot (C) demonstrates a summary of significant alterations of cardiac parameters in term fetuses with HLHS (pink) and neonates with HLHS at 6 hours (teal), 24 hours (blue), and 48 hours (purple) after birth compared with normal fetal and neonatal Ctrl (green). Fetal and neonatal Ctrl presented as a value of 1.