During the 4-year time interval of 2005 through 2008, the Society of Thoracic Surgeons Congenital Heart Surgery Database documented data about 2882 operations to repair atrioventricular (AV) canal defects: partial, 623 (21.5%); intermediate, 342 (11.8%);. complete, 1917 (66.3%). Mean age at complete repair (years) was as follows: partial, 6.1; intermediate, 2.9; complete, 0.6. Median age at complete repair (years) was as follows: partial, 2.6; intermediate, 0.9; complete, 0.4. Down syndrome was present in 1767 patients (61.1%). Debanding of the pulmonary artery was rarely performed: partial, 1 (0.2%); intermediate, 0 (0.0%); complete, 66 (3.4%). Deep hypothermic circulatory arrest was rarely used: partial, 6 (1.0%); intermediate, 5 (1.5%); complete, 52 (2.7%). Discharge mortality was low: partial, 2 (0.3%); intermediate, 3 (0.9%); complete, 38 (2.0%). Atrioventricular block requiring permanent pacemaker occurred but was uncommon: partial, 6 (1.0%); intermediate, 2 (0.6%); complete, 29 (1.5%). Unplanned reoperation prior to hospital discharge occurred in 3.9% of complete AV canal repairs. The sternum was left open in 3.0% of complete AV canal repairs. Postoperative cardiac arrest occurred in 1.9% of complete AV canal repairs. Mean postoperative length of stay (days) was as follows: partial, 5.2; intermediate, 7; complete, 13.1. Median postoperative length of stay (days) was as follows: partial, 4; intermediate, 4; complete, 7. This review of data from the Society of Thoracic Surgeons Congenital Heart Surgery Database allows for unique documentation of patterns of practice and outcomes. From this review, we learned that 98% to 99% of patients survive complete repair of AV canal and 96% to 97% survive complete repair of AV canal with no major complications.

Background Delirium is acute brain dysfunction associated with serious illness. Emerging data indicate that delirium occurs in greater than 20% of children in pediatric intensive care units. Cardiac bypass surgery is a known risk factor for delirium in adults, but has never been systematically studied in pediatrics. Objectives To describe the incidence of delirium in pediatric patients after cardiac bypass surgery, and explore associated risk factors and effect of delirium on in-hospital outcomes. Design Prospective observational single-center study. Setting Fourteen-bed pediatric cardiothoracic intensive care unit (PCICU). Patients One hundred and ninety four consecutive admissions following cardiac bypass surgery, age one day to 21 years. Interventions Subjects were screened for delirium daily using the Cornell Assessment of Pediatric Delirium. Measurements and Main Results Incidence of delirium in this sample was 49%. Delirium most often lasted 1–2 days, and developed within the first 1–3 days after surgery. Age less than two years, developmental delay, higher RACHS-1 score, cyanotic disease, and albumin less than three were all independently associated with development of delirium in a multivariable model (all p values <0.03). Delirium was an independent predictor of prolonged ICU LOS, with patients who were ever delirious having a 60% increase in ICU days compared to patients who were never delirious (p<0.01). Conclusions In our institution, delirium is a frequent problem in children after cardiac bypass surgery, with identifiable risk factors. Our study suggests that cardiac bypass surgery significantly increases children’s susceptibility to delirium. This highlights the need for heightened, targeted delirium screening in all PCICUs to potentially improve outcomes in this vulnerable patient population.

Background Re-coarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt (MBTS) or right ventricle-pulmonary artery shunt (RVPAS). We sought to determine incidence of re-coarctation, risk factors and outcomes in the SVR trial. Methods and Results Re-coarctation was defined by intervention, either catheter-based or surgical. Univariate analysis and multivariable Cox proportional hazard models were performed adjusting for center. Of the 549 SVR subjects, 97 (18%) underwent 131 interventions (92 balloon aortoplasty; 39 surgical) for re-coarctation at median age 4.9 months (range: 1.1–10.5). Intervention typically occurred at pre-stage II catheterization (n=71, 54%) or at stage II surgery (n=38, 29%). In multivariable analysis, re-coarctation was associated with the shunt type in place at the end of the Norwood procedure (HR 2.0 for RVPAS vs. MBTS, p=0.02), and Norwood discharge peak echo-Doppler arch gradient (HR 1.07 per 1 mmHg, p<0.01). Subjects with re-coarctation demonstrated comorbidities at pre-stage II evaluation including higher pulmonary arterial pressures (15.4±3.0 vs. 14.5±3.5 mm Hg; p=0.05), higher pulmonary vascular resistance (2.6±1.6 vs. 2.0±1.0 WU × m2; p=0.04) and increased echocardiographic volumes (end-diastolic volume: 126±39 vs. 112±33 ml/BSA1.3; p=0.02). There was no difference in 12-month post-randomization transplant-free survival between those with and without re-coarctation (p=0.14). Conclusions Re-coarctation is common after Norwood and contributes to pre-stage II comorbidities. Although with intervention there is no associated increase in 1-year transplant/mortality, further evaluation is warranted to evaluate effects of associated morbidities.

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In this descriptive analysis, data in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database pertaining to patients who underwent reoperative cardiac surgery were analyzed. Practice patterns and outcomes are described. Reoperative cardiac surgery for congenital heart disease is common, with one third of index operations in the database occurring subsequent to prior cardiothoracic operation(s) performed on cardiopulmonary bypass. This analysis suggests that a history of previous cardiac surgery does not independently confer a significant incremental risk of operative mortality, but that patients with greater number of previous operations appear to be at higher risk.

Objective A recent STS database study showed that low weight (<2.5 kg) at surgery was associated with a high operative mortality (16%). We thought to assess outcomes after cardiac repair in patients <2.5 kg as compared to 2.5-4.5kg in an institution with a dedicated neonatal cardiac program; and to determine the potential role played by prematurity, STAT risk categories, uni/biventricular pathway, and timing of surgery. Methods We analyzed outcomes (hospital mortality, early reintervention, postoperative length of stay (postopLOS), mortality (at last follow-up) in patients <2.5kg at time of surgery (n=146; group1) and 2.5-4.5kg (n=622; group2), who underwent open or closed cardiac repairs from January 2006 to December 2012 at our institution. The statistical analysis was stratified by prematurity, STAT risk categories, uni/biventricular pathway and “usual”/“delayed” timing of surgery. A uni/multivariate risk analysis was performed. Mean follow-up was 21.6±25.6 months. Results Hospital mortality in group 1 was 10.9% (n=16) vs. 4.8% (n=30) in group 2 (p=0.007). PostopLOS and early un-planned reintervention rates were similar between the two groups. Late mortality in group 1 was 0.7% (n=1). In Group 1, early outcomes were independent of STAT risk categories, uni/biventricular pathway or timing of surgery, as opposed to group 2. Lower gestational age at birth was an independent risk factor for early mortality in group 1. Conclusions A dedicated multidisciplinary neonatal cardiac program yields good outcomes for neonates and infants <2.5kg independently of STAT risk categories and uni/biventricular pathway. Lower gestational age at birth was an independent risk factor for hospital mortality.

Background Pediatric patients with atrioventricular valve disease have limited options for prosthetic valve replacement in sizes <15 mm. Based on successful experience with the stented bovine jugular vein graft (Melody valve) in the right ventricular outflow tract, the prosthesis has been modified for surgical valve replacement in pediatric patients with atrioventricular dysfunction with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. Methods and Results A multicenter, retrospective cohort study was performed among patients who underwent atrioventricular valve replacement with Melody valve at 17 participating sites from North America and Europe, including 68 patients with either mitral (n=59) or tricuspid (n=9) replacement at a median age of 8 months (range, 3 days to 13 years). The median size at implantation was 14 mm (range, 9-24 mm). Immediately postoperatively, the valve was competent with low gradients in all patients. Fifteen patients died; 3 patients underwent transplantation. Nineteen patients required reoperation for adverse outcomes, including valve explantation (n=16), left ventricular outflow tract obstruction (n=1), permanent pacemaker implantation (n=1), and paravalvular leak repair (n=1). Twenty-five patients underwent 41 episodes of catheter-based balloon expansion, exhibiting a significant decrease in median gradient ( P<0.001) with no significant increase in grade of regurgitation. Twelve months after implantation, cumulative incidence analysis indicated that 55% of the patients would be expected to be free from death, heart transplantation, structural valve deterioration, or valve replacement. Conclusions The Melody valve is a feasible option for surgical atrioventricular valve replacement in patients with hypoplastic annuli. The prosthesis shows acceptable short-term function and is amenable to catheter-based enlargement as the child grows. However, patients remain at risk for mortality and structural valve deterioration, despite adequate early valvular function. Device design and implantation techniques must be refined to reduce complications and extend durability. Clinical Trial Registration URL: https://www.clinicaltrials.gov. Unique identifier: NCT02505074.