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Single Ventricle Anatomy Should Not Be An Absolute Contraindication To Cardiac Repair In The Setting Of Congenital Diaphragmatic Hernia
Kylie I. Holden, MD MS1, Michael C. Scott, MD1, Ashley H. Ebanks, NP1, Amir M. Khan, MD1, Michael McMullan, MD2, Anthony Johnson, DO1, Charles S. Cox, MD1, Matthew T. Harting, MD MS1, Damien J. LaPar, MD MSc1.
1University of Texas at Houston, Houston, TX, USA, 2Seattle Children's Hospital, Seattle, WA, USA.

Objective Surgical decision making for congenital heart disease (CHD) with congenital diaphragmatic hernia (CDH) remains challenging, especially in the setting of single ventricle anatomy. The purpose of this study was to assess the surgical outcomes for this population.Methods9,261 patients were evaluated within the CDH Study Group registry (108 centers, 2007-2022), including 222 patients undergoing diaphragm+cardiac repair, 20 patients had single ventricle anatomy. CHD operations were stratified by STAT category. CDH anatomy was categorized by defect size (A-D, smallest to largest). One sample t test, Kruskal-Wallis Test, and Chi-Square Test were employed. ResultsOf the 20 patients, 40.0% underwent STAT4 and 60.0% STAT5 operations. The most common defect size was a type C (60.0%, n=12), followed by A (20.0%, n=4), B (15.0%, n=3), and D (5.0%, n=1). Chromosomal anomaly prevalence was 15.0% (n=3), and the incidence of ECLS was 15.0% (n=3). Mortality was 25.0%. The mean number of days until diaphragm repair was 11.1±14.1 days and cardiac repair was 35.7±39.5 days. When compared to the mean number of days until diaphragm and cardiac repair for the overall cohort (13.9±27.9 days, 80.9±86.5 days, respectively), there was no significant difference in time to diaphragm repair (p=0.65), however, there was a significant difference in time to cardiac repair (p=0.02). ConclusionsSurgical palliation for single ventricle anomalies in the setting of CDH may be performed with lower mortality than previously reported.



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