Effects of universal critical CHD screening of neonates at a mid-sized California congenital cardiac surgery centre.

INTRODUCTION: CHD affects over 1 million children in the United States. Studies show decreased mortality from CHD with newborn cardiac screening. California began a screening programme on 1 July, 2013. We evaluated the effect of mandatory screening on surgical outcomes at Loma Linda University Children's Hospital since 1 July, 2013. METHODS: We evaluated all infants having congenital heart surgery at Loma Linda University Children's Hospital between 1 July, 2013 and 31 December, 2018. Primary target diagnoses include hypoplastic left heart syndrome, pulmonary atresia with intact ventricular septum, tetralogy of Fallot, total anomalous pulmonary venous return, transposition of the great arteries, tricuspid atresia, and truncus arteriosus. Secondary target diagnoses include aortic coarctation, double outlet right ventricle, Ebstein anomaly, interrupted aortic arch, and single ventricle. Patients were stratified by timing of diagnosis (pre-screen, screen positive, and screen negative). Primary end points were post-operative length of stay, operative mortality, absolute mortality, and actuarial survival. RESULTS: The cohort included 274 infants. Of these, 79% were diagnosed prior to screening (46% prenatally). Only 38% of those screened were positive, with 13% of the cohort having a "missed diagnosis." CONCLUSIONS: Primary targets were more likely to be diagnosed by screening (53%), while secondary targets were unlikely to be diagnosed by screening (10%) (p = 0.004). Outcomes such as length of stay, operative mortality, and actuarial survival were not different based on timing of diagnosis (p > 0.05). Despite late diagnosis, those not diagnosed until after screening did not have adverse outcomes.

Contemporary outcomes of complete atrioventricular septal defect repair: analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

OBJECTIVE: Contemporary outcomes data for complete atrioventricular septal defect (CAVSD) repair are limited. We sought to describe early outcomes of CAVSD repair across a large multicenter cohort, and explore potential associations with patient characteristics, including age, weight, and genetic syndromes. METHODS: Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database having repair of CAVSD (2008-2011) were included. Preoperative, operative, and outcomes data were described. Univariate associations between patient factors and outcomes were described. RESULTS: Of 2399 patients (101 centers), 78.4% had Down syndrome. Median age at surgery was 4.6 months (interquartile range, 3.5-6.1 months), with 11.8% (n = 284) aged ≤ 2.5 months. Median weight at surgery was 5.0 kg (interquartile range, 4.3-5.8 kg) with 6.3% (n = 151) < 3.5 kg. Pulmonary artery band removal at CAVSD repair was performed in 122 patients (4.6%). Major complications occurred in 9.8%, including permanent pacemaker implantation in 2.7%. Median postoperative length of stay (PLOS) was 8 days (interquartile range, 5-14 days). Overall hospital mortality was 3.0%. Weight < 3.5 kg and age ≤ 2.5 months were associated with higher mortality, longer PLOS, and increased frequency of major complications. Patients with Down syndrome had lower rates of mortality and morbidities than other patients; PLOS was similar. CONCLUSIONS: In a contemporary multicenter cohort, most patients with CAVSD have repair early in the first year of life. Prior pulmonary artery band is rare. Hospital mortality is generally low, although patients at extremes of low weight and younger age have worse outcomes. Mortality and major complication rates are lower in patients with Down syndrome.

Blake drains: a novel method of chest drainage after extracardiac fontan operation with autologous pericardium.

BACKGROUND: Pleural effusions after Fontan palliation remain a cause of increased length of stay, cost, and morbidity. We report our experience with Blake drains (BDs) and the outpatient pediatric pleural drain pathway after Fontan operation. METHODS: A retrospective chart review was performed on all patients who underwent extracardiac lateral tunnel (ECLT) Fontan operation with pedicled autologous pericardium. Patients with prolonged pleural drainage were analyzed for predisposing factors. RESULTS: From March 1995 to December 2009, 162 patients (92 male, 56.8%) underwent ECLT Fontan operation. The median age at the time of Fontan operation was 30.9 months; the median weight was 12.9 kg. The median hospital stay was 4 days, and the median pleural drain requirement was 13 days. Prolonged pleural effusions occurred in 59 patients (36.4%), with prolonged cardiopulmonary bypass time identified as the only significant risk factor (p=0.04). Sixty patients (37%) were readmitted within 30 days of operation, with effusion requiring additional pleural drainage (n=41, 68.3%), infection (n=8, 13.3%), or a combination of the two (n=3, 5%) being the most common reason. There were two early deaths, neither of which was associated with BD malfunction. The BD clinical pathway for ECLT Fontan operation reduced our institutional cost to about $38,000 per patient, which represents a significant savings compared with traditional management with extended hospital stay after Fontan operation. CONCLUSIONS: Silicone BDs are safe and effective after ECLT Fontan operation. Hospital length of stay and cost can be significantly decreased when these drains are used with appropriate family involvement and close outpatient surveillance.