Impact of Left-sided Lesions in Pediatric Patients Having Surgery for Ebstein Anomaly

• Published in: Pediatrics (Evanston) Vol. 144; no. 2_MeetingAbstract; p. 294
• Main Authors: Haggerty, Brielle J., Van Dorn, Charlotte S., Anderson, Heather N., Dearani, Joseph A., Wackel, Philip L., Cetta, Frank, Johnson, Jonathan N.
• Format: Journal Article
• Language: English
• Published: Evanston American Academy of Pediatrics 01-08-2019
• Subjects: Aortic valve; Children; Complications; Demographics; Heart; Heart surgery; Lactic acid; Males; Mechanical ventilation; Mitral valve; Mitral valve prolapse; Morbidity; Mortality; Orifices; Patients; Pediatrics; Tricuspid valve; Ventilation; Ventilators; Ventricle
• ISSN: 0031-4005; 1098-4275
• DOI: 10.1542/peds.144.2MA3.294

Purpose: Pediatric patients undergoing tricuspid valve intervention for Ebstein anomaly (EA) are at risk for post-operative morbidity and mortality. The impact of left-sided abnormalities (LSA), including left ventricular non-compaction (LVNC) as well as abnormalities of the mitral and aortic valves, on early cardiac post-operative outcomes is unknown. We sought to determine factors that impact early post-operative outcomes in pediatric patients undergoing cone repair (CR) for EA. Methods: We performed a retrospective review of all children 1 to 18 years of age undergoing CR for EA at our institution between 6/1/2007-6/20/2017. We assessed demographic and intraoperative factors, and their relationship to outcomes in patients with and without LSA. Data were analyzed using a student t-test for mean values and 2-tail Fisher exact for categorical data. Results: One hundred sixty-eight patients (mean age 9.1 ± 4.9 years, 54% males) had CR for EA. Twenty-four (14%) had LSA: LVNC (n=1), mitral valve abnormality (n=18), bicuspid aortic valve (n=5). Mitral valve abnormalities included mitral valve prolapse (n = 11), cleft mitral valve (n = 2), double orifice mitral valve (n = 1), and parachute mitral valve (n = 1). Mean cardiopulmonary bypass time was 113 ±26 minutes and mean aortic cross clamp time 83 ± 24 minutes. During the post-operative course, mean length of mechanical ventilation was 1 ± 1 day, mean ICU length of stay (LOS) 4 ± 3 days, and mean hospital LOS 8 ±4 days. Postoperative complications included tricuspid valve reoperation (n=1), post-operative ECMO (n=1), delayed sternal closure (n= 2). There was no postoperative mortality. Six patients (25%) had clinically significant arrhythmias requiring outpatient treatment. LSA was associated with longer CPB (113 ±26 minutes versus 96 ±26 minutes, 0.004). LSA was not associated with longer mechanical ventilation time, ICU or hospital LOS, or increased postoperative complications. Peak lactate was 8 ±1 for patients with LSA versus 7 ±0 for patients without LSA (p = 0.11). Conclusion: LSA are observed in 14% of children with EA. The majority had an abnormality of the mitral valve (75%) usually mitral valve prolapse. The presence of LSA in pediatric EA did increase cardiopulmonary bypass time; however, no significant increase in LOS or post-operative morbidity was appreciated.