Objective Congenital heart disease is commonly a manifestation of Genetic Conditions (GC). Surgery and/or ECMO were withheld in the past from some patients with GC. We hypothesized that surgical care of children with GC has increased over the last decade but their cardiac ECMO use remains lower and mortality greater. Design Retrospective Cohort Study Setting Patients admitted to the Pediatric Health Information System database ≤18 years old with cardiac surgery during 2003–14. GC identified by ICD9 codes were grouped as: Trisomy 21 (T21), Trisomy 13 or 18 (T13/18), 22q11 deletion, and all “other” GC and compared to patients without GC Patients A total of 95,253 patients met study criteria, no GC (85%), T21 (10%), T13/18 (0.2%), 22q11 deletion (1%), and “others” (5%). Interventions None Measurements and Main Results Annual surgical cases did not vary over time. Compared to patients without GC, T21 patient ECMO use was just over half (OR 0.54), but mortality with and without ECMO were similar. In T13/18 patients, ECMO use was similar to those without GC, but all 5 treated with ECMO died. 22q11 patients compared to those without GC had similar ECMO use, but greater odds of ECMO mortality (OR 3.44). “Other” GC had significantly greater ECMO use (OR 1.22), mortality with ECMO (OR 1.42) and even greater mortality odds without (OR 2.62). Conclusions The proportion of children undergoing cardiac surgery who have GC did not increase during the study. Excluding T13/18, all groups of GCs received and benefited from ECMO, although ECMO mortality was greater for those with 22q11 deletion and “other” GC.

BackgroundCardiac mortality and morbidity remain signi cant in children on chronic hemodialysis (HD). Multiple markers by speckle tracking echocardiography (STE) are predictive of cardiac events in adults, but data in children are limited; ventricular diastolic strain (Ds) and left atrial strain (LAS) have not been reported.The aim of this study was to investigate intradialytic changes in ventricular and atrial function using STE in children. MethodsSecondary analysis of a prospective descriptive study of children with structurally normal hearts and HD vintage > 3 months, using echocardiography performed prior to, during, and after HD. STE was analyzed using GE EchoPAC. Left ventricular (LV) global longitudinal strain (GLS), strain rate (Sr), and mechanical dispersion index (MDI) were calculated as the average from 3 apical views; Ds and Sr were obtained from 4-chamber tracing; LAS and Sr were assessed during atrial contraction, reservoir, and conduit. Intradialytic changes in 2D STE markers of ventricular function and LAS and their association with blood volume (BV) removal and blood pressure (BP) change were studied. ResultsA total of 15 children were enrolled at a median age of 12 years (IQR 8, 16) and median HD vintage of 13 months (IQR 9, 25). GLS was impaired during HD (-15.8 ± 2.2% vs -19.9 ± 1.9%, p < 0.001). Post-HD GLS was associated mean BP decrease (coe cient = 0.62, p = 0.01) and not with BV removal (coe cient = 0.49, p = 0.06). LV MDI and systolic Sr did not change. LV Ds was progressively impaired (-8.4% (-9.2, -8.0) vs − 11.9% (-13.4, -10.3), p < 0.001). LAS conduit and reservoir worsened at mid-HD and returned to baseline post-HD. Changes in Ds, DSr, LAS, LASr were not associated with BV removal or BP decrease (p > 0.1). ConclusionsIntradialytic LV strain and LAS impairment consistent with subclinical systolic and diastolic dysfunction were observed during HD in children. GLS impairment was associated with BP change, but changes in Ds, DSr, LAS, and LASr were not associated with BP change or BV removal. Longitudinal assessment using these novel non-invasive indices may unfold the effect of cumulative subclinical changes on long-term outcomes in children who are on chronic HD.

Background: Anomalous aortic origin of the left coronary artery (AAOLCA) confers a rare, but significant, risk of sudden cardiac death in children. Surgery is recommended for interarterial AAOLCA, and other subtypes considered benign. We aimed to determine the clinical characteristics and outcomes of 3 AAOLCA subtypes. Methods: All patients with AAOLCA <21 years old were prospectively enrolled (December 2012–November 2020), including group 1: AAOLCA from the right aortic sinus with interarterial course, group 2: AAOLCA from the right aortic sinus with intraseptal course, and group 3: AAOLCA with a juxtacommissural origin between the left and noncoronary aortic sinus. Anatomic details were assessed using computed tomography angiography. Provocative stress testing (exercise stress testing and stress perfusion imaging) was performed in patients >8 years old or younger if concerning symptoms. Surgery was recommended for group 1 and in select cases in group 2 and group 3. Results: We enrolled 56 patients (64% males) with AAOLCA (group 1, 27; group 2, 20; group 3, 9) at median age of 12 years (interquartile range, 6–15). Intramural course was common in group 1 (93%) compared with group 3 (56%) and group 2 (10%). Seven (13%) presented with aborted sudden cardiac death (group 1, 6/27; group 3, 1/9); 1 (group 3) with cardiogenic shock. Fourteen/42 (33%) had inducible ischemia on provocative testing (group 1, 32%; group 2, 38%; group 3, 29%). Surgery was recommended in 31/56 (56%) patients (group 1, 93%; group 2, 10%; and group 3, 44%). Surgery was performed in 25 patients at a median age 12 (interquartile range, 7–15) years; all have been asymptomatic and free from exercise restrictions at median follow-up of 4 (interquartile range, 1.4–6.3) years. Conclusions: Inducible ischemia was noted in all 3 AAOLCA subtypes while most aborted sudden cardiac deaths occurred in interarterial AAOLCA (group 1). Aborted sudden cardiac death and cardiogenic shock may occur in AAOLCA with left/nonjuxtacommissural origin and intramural course, thus also deemed high-risk. A systematic approach is essential to adequately risk stratify this population.

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