Update of How Pediatric Cardiologists Noninvasively Evaluate Patients with Hypoplastic Left Heart Syndrome: 2013 vs. 2023

Noninvasive functional assessment of systemic right ventricles (RV) in hypoplastic left heart syndrome (HLHS) is challenging. This study aimed to compare pediatric cardiologists' current noninvasive imaging practices to those reported in 2013. A web-based survey was distributed to pediatric cardiologists via various listservs which queried timing of echocardiograms during HLHS palliative stages and measurements of RV function. Demographics of 156 participants who completed the 2023 survey were similar to survey participants in 2013 (n = 222). Respondents were mostly male (62%), echocardiographers (48%), in university-based practice (67%) in North America (95%). Echocardiograms were predominantly obtained monthly during interstage I (41%), every 6 months during interstage II (56%), and every year post-Fontan (68%), which is consistent from 2013. Routine cardiac magnetic resonance imaging (cMRI) significantly increased in interstage II (8.2%, 17%) and post-Fontan (24%, 56%) populations, respectively. Qualitative assessment (41%), ejection fraction (EF) by 3D (20%), fractional area change (16%), and RV strain/strain rate (13%) were preferred methods for systolic assessment, whereas a plurality of respondents (41%) did not believe RV diastolic measurements were valid. The largest gap between currently obtained and desired measurements existed for EF by 3D (46.5% points) and RV strain/strain rate (44.5% points). No differences existed between imagers compared to non-imagers. Variability in evaluating HLHS patients continues among pediatric cardiologists compared to 10 years ago. Qualitative assessment remains the primary RV systolic functional evaluation. Providers do not rely on quantitative RV diastolic function assessments in HLHS patients. Use of cMRI is increasing for RV functional analysis.