Cruz, Stephanie MD; Lau, Patricio E. MD; Rusin, Craig PhD; Style, Candace MD; Ocampo, Elena MD; Rhee, Christopher MD; Brady, Kenneth M. MD; Andropoulos, Dean B. MD; Olutoye, Oluyinka O. MBChB, PhD, FACS Author Information

Background. Anatomic lesions are a common cause of decompensation during the interstage period after Norwood stage 1 palliation (S1P). This study describes the risk factors for and outcomes after unplanned surgical and catheter-based interstage cardiac interventions. Methods. Participants in the National Pediatric Cardiology Quality Improvement Collaborative registry discharged from the hospital after S1P between 2008 and 2016 were studied. Variables at S1P, interstage, and at stage 2 palliation (S2P) hospitalizations were examined. Multivariable logistic regression was used to compare those who had an unplanned interstage intervention to those who did not. Results. Of 1994 participants from 60 programs, 343 (17.1%) had at least 1 unplanned interstage intervention. Aortic valve dilation before S1P, longer S1P cardiopulmonary bypass time, pulmonary artery stent placement between S1P and discharge, aortic arch obstruction on the S1P discharge echocardiogram, and lower weight at S1P discharge were independently associated with receiving an unplanned interstage intervention. Interstage mortality between groups was similar at 6%, as was interstage duration. Participants undergoing unplanned interstage interventions were more likely to undergo heart transplant before S2P or deemed to be unsuitable for S2P (7.3% vs 2.7%, P < .001). Conclusions. Unplanned interstage interventions after S1P did not increase interstage mortality, but participants with an unplanned intervention were less likely to progress to S2P. Residual anatomic lesions are risk factors for unplanned interstage interventions. For those with progressive ventricular dysfunction in the presence of arch obstruction by echocardiogram, aortic arch reintervention is warranted. (C) 2019 by The Society of Thoracic Surgeons

BACKGROUND:When performed by cardiologists, hand-held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies.METHODS:Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric cardiologist on the same day as standard echocardiography (SE). Using 4-point scales, bedside HHE assessment of RV systolic function and TR were compared with blinded assessment of offline SE images. Concordance correlation coefficient (CCC) was used to evaluate agreement.RESULTS:Thirty-two HHEs were performed on 15 subjects (Stage I: n = 17 and Stage II: n = 15). Median subject age was 3.4 months (14 days-4.2 years). Median weight was 5.9 kg (2.6-15.4 kg). Bedside HHE assessment of RV systolic function and TR severity had substantial agreement with SE (CCC = 0.80, CCC = 0.74, respectively; P < .001). HHE sensitivity and specificity for any grade of depressed RV systolic function were 100% and 92%, respectively, and were 94% and 88% for moderate or greater TR, respectively. Average HHE scan time was 238 seconds.CONCLUSIONS:HHE offers a rapid, bedside tool for pediatric cardiologists to detect RV systolic dysfunction and hemodynamically significant TR in HLHS.

Background. The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short-and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure. Methods. All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan take-down or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both). Results. The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n = 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival. Conclusions. Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood. (C) 2019 by The Society of Thoracic Surgeons

Purpose: The purpose of this study was to evaluate the characteristics of patients with congenital heart disease (CHD) who developed necrotizing enterocolitis (NEC). Methods: A retrospective review of neonates with CHD at a tertiary care center between January 2006 and January 2016 was performed. Diagnosis of NEC was based on modified Bell's criteria. Patients were grouped by Risk Adjustment for Congenital Heart Surgery (RACHS-1) or by ductal-dependent (DD) lesions that require a patent ductus arteriosus to supply pulmonary or systemic circulation. Results: Of 1811 neonates with CHD, 3.4% (n = 61) developed NEC. Eighteen (30%) of these required surgical management. The rate of NEC among DD patients was 5% (n = 33/653), compared to 2.4% (n = 28/1158) in the non-DD group (p = 0.003). RACHS-1 score > 2 had a higher rate of NEC 6.2% (41/658) compared to RACHS-1 = 2 cases, 1.7% (20/1153) (p = 0.005). DD patients and complex patients with RACHS-1 > 2 were more likely to develop NEC after cardiac surgery. Hypoplastic left heart syndrome patients had a rate of 9% (n = 16/185). Surgical NEC was more prevalent in the non-DD group. Mortality was similar among groups. Conclusion: CHD patients with ductal-dependent lesions or complex cases (RACHS-1 score > 2) have higher rates of NEC than non-ductal-dependent patients or RACHS-1 score of 2 or less. Mortality is similar regardless of ductal dependence, but surgical NEC was more prevalent in non-DD patients. (C) 2018 Elsevier Inc. All rights reserved.

A surge in cortisol levels is seen after surgery with cardiopulmonary bypass (CPB). Based on evidence of attenuation of the cortisol response to repeated stress in other settings, we hypothesized that the magnitude of cortisol increase in children after a second exposure to CPB would be reduced. Serial cortisol levels were measured at three time points after each CPB: immediately (day 0), on the first morning (day 1), and second morning (day 2). Forty-six children underwent two surgeries with CPB during the study period. The mean age (standard deviation) at first and second surgery was 3.5 (6.3) months and 10.4 (9.9) months, respectively. Cortisol levels at the first surgery were 109 (105) µg/dl, 29 (62) µg/dl, and 17 (12) µg/dl on day 0, 1, and 2, respectively; similarly at second surgery, it was 61 (57) µg/dl on day 0 to 20 (16) µg/dl and 11 (10) µg/dl on day 1 and 2, respectively. After log-transformation and adjusting for time interval between surgeries, cortisol levels at the second surgery were lower by 42% on day 0 (p = 0.02), and 46% lower on day 2 (p = 0.02). A second exposure to CPB in children with congenital heart disease is associated with an attenuated cortisol release.

Objective Critical congenital heart disease (CCHD) is a leading cause of death in infants. Newborn screening (NBS) by pulse oximetry allows early identification of CCHD in asymptomatic newborns. To improve readiness of hospital neonatal birthing facilities for mandatory screening in Texas, an educational and quality improvement (QI) project was piloted to identify an implementation strategy for CCHD NBS in a range of birthing hospitals.Study Design Thirteen Texas hospitals implemented standardized CCHD screening by pulse oximetry. An educational program was devised and a tool kit was created to facilitate education and implementation. Newborn nursery nurses' knowledge was assessed using a pre- and posttest instrument.Results The nurses' knowledge assessment improved from 71 to 92.5% (p < 0.0001). Of 11,322 asymptomatic newborns screened after 24 hours of age, 11 had a positive screen, with 1 confirmed case of CCHD. Pulse oximetry CCHD NBS had sensitivity of 100%, specificity of 99.91%, false-positive rate of 0.088%, positive predictive value of 9.09%, and negative predictive value of 100%.Conclusion Our educational program, including a tool kit, QI processes, and standardized pulse oximetry CCHD NBS, is applicable for a range of hospital birthing facilities and may facilitate wide-scale implementation, thereby improving newborn health.

Congenital heart defects involving left-sided lesions (LSLs) are relatively common birth defects with substantial morbidity and mortality. Previous studies have suggested a high heritability with a complex genetic architecture, such that only a few LSL loci have been identified. We performed a genome-wide case-control association study to address the role of common variants using a discovery cohort of 778 cases and 2756 controls. We identified a genome-wide significant association mapping to a 200 kb region on chromosome 20q11 [P= 1.72 × 10-8 for rs3746446; imputed Single Nucleotide Polymorphism (SNP) rs6088703 P= 3.01 × 10-9, odds ratio (OR)= 1.6 for both]. This result was supported by transmission disequilibrium analyses using a subset of 541 case families (lowest P in region= 4.51 × 10-5, OR= 1.5). Replication in a cohort of 367 LSL cases and 5159 controls showed nominal association (P= 0.03 for rs3746446) resulting in P= 9.49 × 10-9 for rs3746446 upon meta-analysis of the combined cohorts. In addition, a group of seven SNPs on chromosome 1q21.3 met threshold for suggestive association (lowest P= 9.35 × 10-7 for rs12045807). Both regions include genes involved in cardiac development-MYH7B/miR499A on chromosome 20 and CTSK, CTSS and ARNT on chromosome 1. Genome-wide heritability analysis using case-control genotyped SNPs suggested that the mean heritability of LSLs attributable to common variants is moderately high ([Formula: see text] range= 0.26-0.34) and consistent with previous assertions. These results provide evidence for the role of common variation in LSLs, proffer new genes as potential biological candidates, and give further insight to the complex genetic architecture of congenital heart disease.

Objectives/Hypothesis: Vocal fold motion impairment (VFMI) is a known sequela of cardiovascular surgery. The gold standard for the evaluation of vocal fold movement is flexible nasolaryngoscopy (FNL). Although safe, FNL does cause measurable physiologic changes. Noxious stimuli in cardiovascular intensive care unit (CVICU) neonates may cause imbalance between the pulmonary and systemic circulations and potentially circulatory collapse. The goals of this project were to determine the accuracy of laryngeal ultrasound (LUS) compared to FNL to identify VFMI in CVICU neonates and compare their physiologic impact. Study Type: Case control. Design: Prospective case-control study. Methods: Forty-six consecutive infants from the CVICU were recruited, 23 with VFMI and 23 without based on FNL findings. They then underwent LUS, which was subsequently reviewed by two pediatric radiologists blinded to the FNL results. Physiologic parameters were recorded before and after the procedures. Data analysis was performed with Cohen's kappa and Wilcoxon signed-rank test. Results: Agreement between LUS and FNL was substantial (kappa = 0.78). Laryngeal ultrasound had a sensitivity of 0.84 and specificity of 0.95 for identifying VFMI. In terms of physiologic impact, LUS caused statistically significantly less change in diastolic blood pressure (P = 0.01), pulse (P = 0.001), and 02 saturation (P = 0.004) compared to FNL. Arytenoid rotation could be seen clearly, and the vocal fold-arytenoid angle in abduction was most predictive of symmetry and movement (P = 0.17). However, it was difficult to determine vocal fold closure patterns with LUS. Conclusion: Laryngeal ultrasound is a comparable to FNL for evaluating vocal fold mobility with less physiologic impact.

Copyright © 2016 by the Society of Critical Care Medicine and Wolters Kluwer Health, Inc. All Rights Reserved.

Objective: To determine the long-term prognosis of children with vocal fold mobility impairment (VFMI) after cardiac surgery, with respect to time to normal feeding and incidence of admissions for pneumonia and feeding difficulties.Methods: A retrospective chart review was conducted of all neonates who had otolaryngology exam after cardiac surgery at a tertiary children's hospital from May 2007 to May 2008. Charts were reviewed for demographics, type of cardiac surgery, vocal fold mobility, diet at time of discharge and at last follow-up, time to full oral feeding, and hospital admissions.Results: There were a total of 94 patients included in the study, 17 of whom had VFMI. While significantly more patients with VFMI required modified diet at discharge, 48% compared to 19% of patients with normal vocal fold mobility; there was no statistically significant difference in time to regular diet on long-term follow-up, 0.8 years (VFMI) compared to 0.4 years (normal vocal fold mobility). Of the 25 patients with modified diet or gastrostomy tube at discharge, 52% returned to full feeds within a year. There was no difference in hospitalizations for pneumonia in patients with or without VFMI. However in patients with VFMI, 35% required readmission for feeding difficulty or poor weight gain compared to only 5% in the infants with normal vocal fold mobility.Conclusion: After neonatal cardiac surgery, there do not appear to be long-term effects of VFMI with regards to readmission for pneumonia. However, there is an increased risk for hospitalization with respect to feeding difficulties in those neonates with VFMI. The overall prognosis for time to oral feeding is good. (C) 2016 Published by Elsevier Ireland Ltd.

Background.Children with SV physiology require prolonged hospitalizations and multiple surgeries during their first year of life.There are limited data on the incidence of infections and associated outcomes in these patients.The objective of this study was to characterize the microbiology, associated risk factors, and clinical outcomes of SV patients at TCH during their first year of life.Methods.We completed a retrospective chart review of infants with SV physiology born between 12/2009 and 4/2015.Results.Of the 127 infants with SV physiology 64% had hypoplastic left heart syndrome, 16% unbalanced atrioventricular canal, 12% double outlet right ventricle, 6% Shone's complex, and 2% double inlet left ventricle.Sixty-nine patients (54%) were male.124 patients (98%) underwent stage one palliation and 99 (78%) underwent

Copyright © 2016 by the Society of Critical Care Medicine and Wolters Kluwer Health, Inc. All Rights Reserved.

Background Feeding dysfunction occurs commonly in infants with single ventricle heart disease and impacts growth and long-term outcomes. Little evidence exists to guide safe feeding in this population. This study surveyed centers participating in the National Pediatric Cardiology Quality Improvement Collaborative to assess prevailing feeding practices amongthose caring for single ventricle neonates. Methods Web-based survey of 56 pediatric cardiac surgical centers was conducted. Questions addressed peri-operative feeding approaches and responses were presented and analyzed descriptively. Results Of 56 centers, 46 (82%) completed a survey. Preoperative feeding was common in single ventricle infants (30/46; 65%), routes varied. Centers who did not feed infants preoperatively cited the risk of necrotizing enterocolitis (16/16; 100%), presence of umbilical artery catheter (12/16; 75%), and prostaglandin infusion (9/16; 56%) as main concerns. 67% of centers reported no specific vital sign thresholds for withholding enteral feedings. In the postoperative period, most centers used an "internal guideline" (21/46; 46%) or an "informal practice" (15/46; 33%) to determine feeding readiness. Approaches to findings were significantly different among centers. About 40% of centers did not send patients home with feeding tubes, and there was no clear consensus between preferred feeding tube modality at discharge. Conclusion Considerable variation exists in feeding practices for infants with single ventricle congenital heart disease among 46 centers participating in a quality improvement collaborative. Although most centers generally feed infants preoperatively, feeding practices remain center-specific. Variability continues in the immediate post-operative and interstage periods. Further opportunities exist for investigation, standardization and development of best-practice feeding guidelines.

Objective: The study objective was to evaluate risk factors for poor weight gain in infants with hypoplastic left heart syndrome after stage 1 palliation.Methods: We reviewed all term infants with hypoplastic left heart syndrome who had stage 1 palliation and stage 2 palliation at Texas Children's Hospital between 2000 and 2011 ( n = 120). Predictor variables included age at stage 1 palliation, intensive care unit factors, calories delivered, and echocardiographic findings. Outcome variables included weight for age Z scores at hospital discharge, stage 2 palliation, and change in weight for age Z scores between stage 1 palliation and hospital discharge.Results: Complete nutritional data were available for 47 of 120 patients. Median total parenteral nutrition duration was 6 days (range, 1-43 days), and median intensive care unit calories delivered was 53.9 kcal/kg/d (range, 22.3-119.6 kcal/kg/d). Before hospital discharge, the median caloric intake was 106.7 kcal/kg/d ( range, 70.0-152.0 kcal/kg/d). Median weight for age Z scores was -0.59 (range, -3.6 to 0.5) at stage 1 palliation, - 1.62 ( range, -4.5 to -0.1) at intensive care unit transfer, and -1.81 (range, -4.9 to - 0.5) at hospital discharge. A total of 46 of 47 patients had a negative change in weight for age Z scores between stage 1 palliation and hospital discharge, with a median change of -1.14 (range, -2.3 to 0.6). Change in weight for age Z scores from stage 1 palliation to discharge was directly associated with calories delivered and indirectly associated with hospital length of stay and moderate tricuspid regurgitation ( P < .001).Conclusions: Postoperative nutrition fails to meet the needs of infants with hypoplastic left heart syndrome despite increased focus on nutritional support. Modifiable factors (eg, nutritional intake) and hemodynamic factors (eg, tricuspid regurgitation) may play roles in the poor weight gain of these infants.

Objectives: The postoperative cortisol profile and its association with early outcomes are poorly understood in neonates undergoing surgery for complex congenital heart disease. We investigated the postoperative profile of cortisol and its relationship with the clinical course in a cohort of newborns after stage-1 palliation for hypoplastic left heart syndrome.Design: Prospective observational study.Setting: Pediatric cardiovascular ICU at a tertiary children's hospital.Subjects: Twenty-three neonates after stage-1 palliation for hypoplastic left heart syndrome between 2009 and 2011.Interventions: None.Measurements and Main Results: Three serial measurements of total serum cortisol after surgery. The first measurement was taken immediately after surgery and the second and thirdon the first and second postoperative mornings. The median weight of the infants was 3.0 kg (2.7-3.4 kg), and the age at surgery was 7 days (6-9 d). The median (25th-75th percentile) cortisol levels at admission, day 1, and day 2 were 96.2 g/dL (51.1-112 g/dL), 17.3 g/dL (9.7-25.1 g/dL), and 10 g/dL (6.5-17 g/dL), respectively (p < 0.0001 between admission and day 1). Higher cortisol was associated with greater morbidity, including the need for preoperative ventilation, increased total duration of ventilation, duration of inotropic support, and hospital length of stay.Conclusions: Cortisol levels fell significantly over the first 24 hours after stage-1 palliation for hypoplastic left heart syndrome. A higher postoperative cortisol was associated with increased postoperative morbidity, which warrants further investigation.