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

Amna Qasim; Tam T Doan; Betul Yilmaz Furtun; Ziyad Binsalamah; Iki Adachi; Shaine A Morris

doi:10.1002/pd.6539

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

Prenat Diagn. 2024 Mar 6. doi: 10.1002/pd.6539. Online ahead of print.

Authors

Amna Qasim^{1

2}, Tam T Doan¹, Betul Yilmaz Furtun¹, Ziyad Binsalamah^{1

3}, Iki Adachi^{1

3}, Shaine A Morris¹

Affiliations

¹ The Lillie Frank Abercrombie Section of Cardiology, Texas Children's Hospital, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA.
² Department of Pediatric Cardiology, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.
³ Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA.

PMID: 38447986
DOI: 10.1002/pd.6539

Abstract

Objectives: A 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.

Methods: This 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).

Results: Of 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).

Conclusion: L-cor in fetal HLHS is associated with increased mortality. Recognition of this finding is important for prognostication and atrial-septal-intervention planning.