Right ventricular (RV) failure is a major cause of morbidity and mortality in patients with hypoplastic left heart syndrome (HLHS), but the longitudinal course of RV volumes through staged palliation (SP) has not been previously investigated. The aim of this study was to evaluate RV volume and function longitudinally through SP of HLHS using real-time three-dimensional echocardiography.

A total of 18 subjects with HLHS were prospectively studied at four time points from diagnosis through stage 2 (SP2). Real-time three-dimensional echocardiographic full-volume data sets were acquired in high–frame rate mode with electrocardiographic gating. Volumetric and functional analyses were performed using a semiautomatic contour detection algorithm. Eighteen age-matched and sex-matched normal infants (aged 0–6 months) were studied at comparable time points as controls.

Presurgical examinations (pre–stage 1 [SP1]; n = 18) were performed at a mean age of 4 days, post-SP1 examinations (n = 17) at a mean age of 20 days, pre-SP2 examinations (n = 14) at a mean age of 4.6 months, and post-SP2 examinations (n = 14) at a mean age of 5.5 months, constituting a total of 63 examinations. The mean values of RV end-diastolic volume indexed to body surface area (EDVi) at the four time points were 87 ± 30, 104 ± 39, 112 ± 34, and 102 ± 35 mL/m², respectively. There was an increase in EDVi (P = .024) from pre-SP1 to post-SP1 but no significant change between post-SP1 and pre-SP2. The decrease in EDVi after SP2 did not reach statistical significance. Mean RV ejection fractions (EFs) were 50 ± 5%, 45 ± 5%, 46 ± 5%, and 38 ± 4%, respectively. There was a trend toward decreasing EF throughout SP, with statistically significant decreases from pre-SP1 to post-SP1 (P = .003) and from pre-SP2 to post-SP2 (P < .001). In normal infants, the mean RV EDVi was 50 ± 10 mL/m² (approximately half that of patients with HLHS), and the mean EF was 51 ± 3%. There was good interobserver agreement for EDVi, end-systolic volume indexed to body surface area, and EF.

Real-time three-dimensional echocardiography is a reproducible means for evaluating RV volumes and EFs in patients with HLHS. Indexed RV diastolic volume remains stable to slightly increased, and RV EF deteriorates as the first two stages of surgical palliation are accomplished. The findings of this study highlight the adverse physiology of HLHS, which deteriorates even among early survivors despite SP.