Kabuki-Syndrome and Congenital Heart Disease-A Twenty-Year Institutional Experience

Background: Patients with genetic syndromes who undergo surgery to correct congenital heart defects can be at risk for increased morbidity or mortality. Surgical outcomes and postoperative courses following congenital heart surgery in patients with Kabuki-Syndrome (KS) have not been well studied. Objectives: The purpose of this study was to describe the postoperative courses and associated outcomes in the largest set of KS patients undergoing congenital heart surgery to date. Methods: Patients with a confirmed molecular diagnosis of KS and a diagnosis of a CHD admitted to Texas Children's Hospital between January 1, 2000 and January 1, 2020 were included (n = 20). Demographics and medical histories were collected from the hospitals' electronic health records. Results: Of 20 patients identified with KS and a CHD, 15 required surgical correction of their congenital cardiac malformation. Median age and weight at the time of surgery was 2 months and 4.1 kg, respectively. Median duration of hospital stay was 49 days for all surgeries and 151 days for the Norwood procedure. Postoperative infections and pleural effusions were detected and treated in 45.8% and 50% of patients, respectively. There was no in-hospital mortality for any surgery. Median follow up time was 5.6 years; survival at 6 years was 94%. Conclusions: Although KS patients seem to be at increased risk for a more complicated, prolonged postoperative course than that of patients without a genetic syndrome, patients with a diagnosis of a CHD and KS do not appear to be at increased risk of mortality following congenital heart surgery.