Prenatally diagnosed coronary artery abnormalities in hypoplastic left heart syndrome are associated with a higher probability of heart transplantation

• Published in: Prenatal diagnosis Vol. 42; no. 11; pp. 1458 - 1465
• Main Authors: Rueda‐de‐Leon, Elena, Killen, Stacy A. S., Soslow, Jonathan H., McHugh, M. Kelly, Fuchs, Sarah R., Kavanaugh‐McHugh, Ann
• Format: Journal Article
• Language: English
• Published: Charlottesville Wiley Subscription Services, Inc 01-10-2022
• Subjects: Abnormalities; Coronary artery; Coronary vessels; Death; Diagnosis; Echocardiography; Fetuses; Heart; Heart transplantation; Mortality; Prenatal diagnosis; Survival analysis; Transplantation; Ultrasonic imaging; Ventricle
• ISSN: 0197-3851; 1097-0223
• DOI: 10.1002/pd.6227

Objective
Coronary artery abnormalities (CA) occur in patients with hypoplastic left heart syndrome (HLHS) and may be associated with higher mortality and heart transplantation (HT). We aimed to determine whether fetuses with HLHS and prenatal CA have a higher risk of death or HT.
Methods
We performed a retrospective review of fetal echocardiograms with HLHS from 2011 to 2018. We excluded fetuses with ventricular septal defects, elective termination, death in utero, planned postnatal non‐intervention, or absent follow‐up data. Presence or absence of CA was determined by review of serial fetal echocardiograms. Survival analysis was used to evaluate the relationship between prenatal CA and death or HT.
Results
Of 86 patients with fetal HLHS, 11 had prenatal diagnosis of CA. Of these, six required HT and five died (one after undergoing HT); only one remains alive without HT. Of those without prenatal CA (n = 75), 25 died and 7 underwent HT. Patients with prenatal diagnosis of HLHS and CA had a significantly increased likelihood of death or HT (p‐value <0.05).
Conclusion
Prenatal diagnosis of CA in our cohort of patients with HLHS was associated with increased risk of death or HT. These data have significance for prenatal counseling and postnatal management.
Key points
What is already known about this topic?
Patients with hypoplastic left heart syndrome (HLHS) may have coronary artery abnormalities that place them at a higher risk for death or heart transplantation.
Coronary artery abnormalities have been identified prenatally in some fetuses with HLHS though the incidence and significance of these findings has not been explored.
What does this study add?
Fetuses with HLHS and suspected coronary artery abnormalities in our cohort had an increased likelihood of heart transplantation, which has significance for prenatal counseling and postnatal management.