Management of Hypertrophic Cardiomyopathy in a Newborn with Dextro-Transposition of the Great Arteries.

Impaired maternal glucose metabolism during pregnancy can have significant effects on the cardiovascular system of the developing fetus. Early in pregnancy the teratogenic effects may lead to structural heart defects, while later in gestation a form of hypertrophic cardiomyopathy can develop due to overgrowth driven by fetal hyperinsulinism. We describe an infant with the uncommon combination of both dextro-transposition of the great arteries and hypertrophic cardiomyopathy. We emphasize the importance of a longitudinal multi-disciplinary approach, from fetal diagnosis to post-operative management, that allowed for an excellent outcome in this rare combination of severe cardiac malformations.

Variability in chest compression rate calculations during pediatric cardiopulmonary resuscitation.

AIM: The mathematical method used to calculate chest compression (CC) rate during cardiopulmonary resuscitation varies in the literature and across device manufacturers. The objective of this study was to determine the variability in calculated CC rates by applying four published methods to the same dataset. METHODS: This study was a secondary investigation of the first 200 pediatric cardiac arrest events with invasive arterial line waveform data in the ICU-RESUScitation Project (NCT02837497). Instantaneous CC rates were calculated during periods of uninterrupted CCs. The defined minimum interruption length affects rate calculation (e.g., if an interruption is defined as a break in CCs ≥ 2 s, the lowest possible calculated rate is 30 CCs/min). Average rates were calculated by four methods: 1) rate with an interruption defined as ≥ 1 s; 2) interruption ≥ 2 s; 3) interruption ≥ 3 s; 4) method #3 excluding top and bottom quartiles of calculated rates. American Heart Association Guideline-compliant rate was defined as 100-120 CCs/min. A clinically important change was defined as ±5 CCs/min. The percentage of events and epochs (30 s periods) that changed Guideline-compliant status was calculated. RESULTS: Across calculation methods, mean CC rates (118.7-119.5/min) were similar. Comparing all methods, 14 events (7%) and 114 epochs (6%) changed Guideline-compliant status. CONCLUSION: Using four published methods for calculating CC rate, average rates were similar, but 7% of events changed Guideline-compliant status. These data suggest that a uniform calculation method (interruption ≥ 1 s) should be adopted to decrease variability in resuscitation science.

Venovenous ECMO Support in an Infant With Single Ventricle Physiology and Catastrophic Pulmonary Venous Abnormalities.

Extracorporeal membrane oxygenation (ECMO) is an accepted treatment modality for life support refractory to conventional efforts in neonates with complex congenital heart lesions. Cannulation for ECMO can be accomplished by venovenous (VV) access where patients receive primarily respiratory support or venoarterial (VA) access which provides complete cardiopulmonary support. VV ECMO delivered by a single-vessel cannulation with a dual-chamber venous cannula allows for respiratory support while the patient remains dependent upon intrinsic cardiac function to support hemodynamics. We present a case of support of a newborn with single ventricle physiology and intraparenchymal near-atresia of the pulmonary veins using VV ECMO.

Interstage Survival for Patients with Hypoplastic Left Heart Syndrome After ECMO.

There is a reported 5-20 % incidence of extracorporeal membrane oxygenation (ECMO) following stage I palliation for hypoplastic left heart syndrome (HLHS). This study compares the interstage mortality of HLHS patients supported with ECMO (HLHS-ECMO) to those who were not supported with ECMO (HLHS-nECMO) using the National Pediatric Cardiology Quality Improvement Initiative database. Patients with HLHS who survived to hospital discharge after stage I palliation were analyzed. HLHS-ECMO patients were compared to HLHS-non-ECMO patients with respect to demographics, surgical variables, and interstage survival. A total of 931 patients were identified in the database. Sixty-six (7.1 %) patients were supported with ECMO during their stage I palliation admission. There were no statistically significant differences between the groups with respect to demographics or anatomic subtype. HLHS-ECMO patients were more likely to have a preoperative risk factor identified (62 vs. 48 %, p = 0.03) or require ECMO prior to stage I palliation (3 vs. 0.5 %, p = 0.03). HLHS-ECMO patients had a significantly higher incidence of death or transplant versus the HLHS-nECMO group (18 vs. 9 %, p = 0.03). Despite survival to discharge, patients with HLHS requiring ECMO after their palliation continue to have an increased risk of death/cardiac transplant versus patients that do not require ECMO. ECMO use is likely a marker for a high-risk patient group. These patients may benefit from closer follow-up during the interstage period.

Multidisciplinary Review of Code Events in a Heart Center.

OBJECTIVE: To identify a cause for clinical deterioration, examine resuscitation efforts, and identify and correct system issues (thus improving outcomes) via a multidisciplinary code-review process soon after cardiopulmonary arrest. METHODS: Retrospective analysis of code events in a tertiary pediatric heart center from September 2010 to December 2013 and review of surgical-cardiac data from January 2010 to December 2013. RESULTS: A multidisciplinary team reviewed 47 code events, 16 of which (34%) were deemed potentially preventable. At least 2 issues were identified during 66% (31/47) of cardiopulmonary arrests reviewed. Key issues identified were related to communication (62%), environment/culture/policy (47%), patient care (including resuscitation, 41%), and equipment (38%). About 60% of reviewed arrests resulted in educational initiatives (eg, mock code, in-service education) and 47% resulted in a new policy or modification of existing policy. Less common were changes in equipment (32%) or modification of staffing needs (11%). Changes most frequently occurred in the unit specific to the event (68%) but some changes occurred throughout the Heart Center (32%) or across the hospital system (13%). Survival to discharge after cardiopulmonary arrest has improved over time (P = .03) to 81% for cardiac surgical patients in our center. CONCLUSION: A multidisciplinary code-review committee can identify deficiencies and lead to educational initiatives and improvements in care. When coupled with a hospital-wide "code blue" review process, these changes may benefit the institution as a whole.

ECMO: Incidence and Outcomes of Patients Undergoing the Hybrid Procedure.

OBJECTIVE: To document the extracorporeal membrane oxygenation (ECMO) incidence and outcome in patients undergoing the hybrid procedure at an institution that routinely performs this procedure. DESIGN: A retrospective chart review on all patients with single ventricle physiology that underwent the hybrid procedure between 7/2002 and 12/2014. Patients were excluded if they underwent the hybrid procedure after 60 days of birth or subsequently underwent a biventricular repair. SETTING: A single center, tertiary pediatric hospital. PATIENTS: One hundred eighty-one patients with single ventricle physiology that underwent the hybrid procedure between 7/2002 and 12/2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We studied a total of 181 patients (105 males: 76 females). Gestational age was 37.8 ± 2.0 weeks and birth weight was 3.0 ± 0.7 kg. Underlying diagnosis was hypoplastic left heart syndrome in 149 patients and other in 32 patients. Age at surgery was 7.8 ± 6.8 days and weight at surgery was 3.1 ± 0.6 kg. Two patients underwent ECMO support after the hybrid procedure. One patient had aortic atresia/mitral atresia and weighed 2.3 kg and the other patient had aortic atresia/mitral stenosis and weighed 2.1 kg at time of surgery. Both patients died. Incidence of ECMO support after hybrid procedure was 1.3% (2/149) for the hypoplastic left heart syndrome patients and 1.1% (2/181) for the entire cohort. CONCLUSION: Mortality in patients who underwent ECMO after the hybrid procedure was higher than reported for the Norwood procedure, however, the incidence of ECMO after hybrid procedure was also significantly lower than reported for the Norwood procedure. Future studies are needed to determine how to improve outcomes in this complex patient population.

Arrhythmias Following Comprehensive Stage II Surgical Palliation in Single Ventricle Patients.

Post-operative arrhythmias are common in pediatric patients following cardiac surgery. Following hybrid palliation in single ventricle patients, a comprehensive stage II palliation is performed. The incidence of arrhythmias in patients following comprehensive stage II palliation is unknown. The purpose of this study is to determine the incidence of arrhythmias following comprehensive stage II palliation. A single-center retrospective chart review was performed on all single ventricle patients undergoing a comprehensive stage II palliation from January 2010 to May 2014. Pre-operative, operative, and post-operative data were collected. A clinically significant arrhythmia was defined as an arrhythmia which led to cardiopulmonary resuscitation or required treatment with either pacing or antiarrhythmic medication. Statistical analysis was performed with Wilcoxon rank-sum test and Fisher's exact test with p < 0.05 significant. Forty-eight single ventricle patients were reviewed (32 hypoplastic left heart syndrome, 16 other single ventricle variants). Age at surgery was 185 ± 56 days. Cardiopulmonary bypass time was 259 ± 45 min. Average vasoactive-inotropic score was 5.97 ± 7.58. Six patients (12.5 %) had clinically significant arrhythmias: four sinus bradycardia, one 2:1 atrioventricular block, and one slow junctional rhythm. No tachyarrhythmias were documented for this patient population. Presence of arrhythmia was associated with elevated lactate (p = 0.04) and cardiac arrest (p = 0.002). Following comprehensive stage II palliation, single ventricle patients are at low risk for development of tachyarrhythmias. The most frequent arrhythmia seen in these patients was sinus bradycardia associated with respiratory compromise.

Snf1-related kinase inhibits colon cancer cell proliferation through calcyclin-binding protein-dependent reduction of ß-catenin.

Sucrose nonfermenting 1 (Snf1)-related kinase (SNRK) is a serine/threonine kinase with sequence similarity to AMP-activated protein kinase (AMPK); however, its function is not well characterized. We conducted a gene array to determine which genes are regulated by SNRK. The array demonstrated that SNRK overexpression increased the levels of genes involved in cell proliferation, including calcyclin-binding protein (CacyBP), a member of the ubiquitin ligase complex that targets nonphosphorylated ß-catenin for degradation. We confirmed that SNRK increased CacyBP mRNA and protein, and decreased ß-catenin protein in HCT116 and RKO colon cancer cells. Furthermore, SNRK inhibited colon cancer cell proliferation, and CacyBP down-regulation reversed the SNRK-mediated decrease in proliferation and ß-catenin. SNRK overexpression also decreased ß-catenin nuclear localization and target gene transcription, and ß-catenin down-regulation reversed the effects of SNRK knockdown on proliferation. SNRK transcript levels were reduced in human colon tumors compared to normal tissue by 35.82%, and stable knockdown of SNRK increased colon cancer cell tumorigenicity. Our results demonstrate that SNRK is down-regulated in colon cancer and inhibits colon cancer cell proliferation through CacyBP up-regulation and ß-catenin degradation, resulting in reduced proliferation signaling. These findings reveal a novel function for SNRK in the regulation of colon cancer cell proliferation and ß-catenin signaling.