Perioperative acute kidney injury is common in infant heart surgery and portends a poor clinical outcome.

NKX2.5 mutations occur in a small percentage of patients with various CHD. Most of the mutations identified in this study were missense, outside the homeodomain, and not associated with AV block. These findings suggest that NKX2.5 mutations in non-homeodomain regions may be important in the development of human structural cardiac defects.

We provide comprehensive multicenter benchmark data regarding rates of HAIs within dedicated pediatric CICUs. We confirm that although rare, HAIs of all types are associated with significant resource utilization and mortality.

OBJECTIVES: Cardiac surgery–associated acute kidney injury occurs commonly following congenital heart surgery and is associated with adverse outcomes. This study represents the first multicenter study of neonatal cardiac surgery–associated acute kidney injury. We aimed to describe the epidemiology, including perioperative predictors and associated outcomes of this important complication. DESIGN: This Neonatal and Pediatric Heart and Renal Outcomes Network study is a multicenter, retrospective cohort study of consecutive neonates less than 30 days. Neonatal modification of The Kidney Disease Improving Global Outcomes criteria was used. Associations between cardiac surgery–associated acute kidney injury stage and outcomes (mortality, length of stay, and duration of mechanical ventilation) were assessed through multivariable regression. SETTING: Twenty-two hospitals participating in Pediatric Cardiac Critical Care Consortium. PATIENTS: Twenty-two–thousand forty neonates who underwent major cardiac surgery from September 2015 to January 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Cardiac surgery–associated acute kidney injury occurred in 1,207 patients (53.8%); 983 of 1,657 in cardiopulmonary bypass patients (59.3%) and 224 of 583 in noncardiopulmonary bypass patients (38.4%). Seven-hundred two (31.3%) had maximum stage 1, 302 (13.5%) stage 2, 203 (9.1%) stage 3; prevalence of cardiac surgery–associated acute kidney injury peaked on postoperative day 1. Cardiac surgery–associated acute kidney injury rates varied greatly (27–86%) across institutions. Preoperative enteral feeding (odds ratio = 0.68; 0.52–0.9) and open sternum (odds ratio = 0.76; 0.61–0.96) were associated with less cardiac surgery–associated acute kidney injury; cardiopulmonary bypass was associated with increased cardiac surgery–associated acute kidney injury (odds ratio = 1.53; 1.01–2.32). Duration of cardiopulmonary bypass was not associated with cardiac surgery–associated acute kidney injury in the cardiopulmonary bypass cohort. Stage 3 cardiac surgery–associated acute kidney injury was independently associated with hospital mortality (odds ratio = 2.44; 1.3–4.61). No cardiac surgery–associated acute kidney injury stage was associated with duration of mechanical ventilation or length of stay. CONCLUSIONS: Cardiac surgery–associated acute kidney injury occurs frequently after neonatal cardiac surgery in both cardiopulmonary bypass and noncardiopulmonary bypass patients. Rates vary significantly across hospitals. Only stage 3 cardiac surgery–associated acute kidney injury is associated with mortality. Cardiac surgery–associated acute kidney injury was not associated with any other outcomes. Kidney Disease Improving Global Outcomes criteria may not precisely define a clinically meaningful renal injury phenotype in this population.

Objectives To understand the effect of tight glycemic control (TGC) on cardiac surgery-associated acute kidney injury (CS-AKI). Design Secondary analysis of data from the Safe Pediatric Euglycemia after Cardiac Surgery (SPECS) trial of TGC vs. standard care Setting Pediatric cardiac intensive care units (ICU) at University of Michigan (UM) C.S. Mott Children’s Hospital and Boston Children’s Hospital (BCH). Patients Children 0–36 months of age undergoing congenital cardiac surgery. Interventions None. Measurements and Main Results CS-AKI was assigned using the Acute Kidney Injury Network criteria with the modification that a >0.1 mg/dL increase in serum creatinine was required to assign CS-AKI. We explored associations between CS-AKI and TGC and clinical outcomes. Of 799 patients studied, CS-AKI occurred in 289 (36%) patients, most of whom had Stage II or III disease (72%). CS-AKI rates were similar between treatment groups (36% vs. 36%, p=0.99). Multivariable modeling showed that patients with CS-AKI were younger (p=0.002), underwent more complex surgery (p=0.005) and had longer cardiopulmonary bypass times (p=0.002). CS-AKI was associated with longer mechanical ventilation and ICU and hospital stays and increased mortality. Patients at UM had higher rates of CS-AKI compared to BCH patients (66% vs. 15%, p<0.001) but UM patients with CS-AKI had shorter time to extubation and ICU and hospital stays compared to BCH. Conclusions TGC did not reduce the CS-AKI rate in this trial cohort. We observed significant differences in CS-AKI rates between the two study sites, and there was a differential effect of CS-AKI on clinical outcomes by site. These findings warrant further investigation to identify causal variation in perioperative practices that affect CS-AKI epidemiology.

After correcting serum creatinine for fluid balance and adjusting for surgical complexity, LFR performs fairly at 2 hours, whereas at 6 hours, LFR is a good AKI predictor. Prospective studies are needed to validate whether diuretic responsiveness predicts AKI.

Interstitial deletion of chromosome 8p23.1 has been reported in patients with congenital heart defects, including atrial and ventricular septal defects, pulmonary stenosis, and complex cyanotic heart defects. GATA4, a zinc-finger transcription factor gene, has been localized to this region. GATA4 interacts with additional transcription factors in the embryogenesis of the primitive heart tube. Mutations in GATA4 are thought to be responsible for the congenital heart defects reported in association with this chromosomal deletion, and several familial point mutations leading to amino acid substitutions have also been identified. Left ventricular noncompaction (LVNC) is a clinically heterogeneous disorder characterized by LV myocardial trabeculations and intertrabecular recesses that communicate with the LV cavity. Patients may be asymptomatic or may present with evidence of severely depressed LV systolic and diastolic function. The LV may be dilated or hypertrophied, and clinical expression may be undulating. Several genetic causes of LVNC have been reported, with variable modes of inheritance, including autosomal dominant and X-linked inheritance, but relatively few responsible genes have been identified. A 12-year-old boy with a history of acute lymphoblastic leukemia, dysmorphic features, and LVNC with preserved LV systolic function was referred to the Cardiovascular Genetics Clinic at our institution. The patient was asymptomatic in terms of cardiovascular function. Chromosome microarray testing revealed an interstitial deletion in the region of 8p23.1 containing GATA4. LVNC has not been reported previously in association with this chromosome deletion. Further investigation into the role of GATA4 in patients with LVNC is warranted.

The predominance of neutrophilic proteinaceous casts and high percentage of positive BAL infectious studies support short-term fibrinolytic and anti-infective therapies in PB in select patients. Flexible bronchoscopy enables safe assessment of cast burden. PCL effectively treats PB and reduces respiratory therapies.