Labyrinthine cor triatriatum sinister in fetal hypoplastic left heart syndrome is associated with poor outcomes

Abstract

AbstractObjectivesA subset of hypoplastic‐left‐heart‐syndrome (HLHS) fetuses have a complex cor‐triatriatum sinister that we named “labyrinthine‐cor (L‐cor)”. We sought to determine the prevalence of L‐cor in HLHS fetuses and hypothesized that it is associated with increased mortality.MethodsThis single‐center retrospective cohort study included all HLHS fetuses from January 2010‐December 2020. Fetuses with other hypoplastic‐left‐heart variants, inadequate images, lack of follow‐up and fetal atrial‐septal interventions were excluded. RAS was defined as the ratio of pulmonary‐vein forward‐to‐reverse velocity‐time‐integral (VTI) ≤ 5 and severe‐RAS defined as VTI‐ratio <3. Kaplan‐Meier survival‐analysis was performed for the primary outcome of transplant‐free survival for 62 weeks after gestational‐age of 30 weeks (∼1 year).ResultsOf the 156 consecutive fetuses with HLHS, 11 (7.7%) had L‐cor and 8/11 (72.7%) of these had RAS. When compared to HLHS‐RAS without L‐cor, fetuses with HLHS‐RAS and L‐cor were less likely to survive to 28 days (87% vs. 62.5%, p = 0.017) and to 1 year (69.6% vs. 25%, p = 0.029). When comparing by survival analysis, fetuses with severe‐RAS with L‐cor had lower survival compared severe‐RAS without L‐cor (p = 0.020).ConclusionL‐cor in fetal HLHS is associated with increased mortality. Recognition of this finding is important for prognostication and atrial‐septal‐intervention planning.