Evaluation and Management of Noncardiac Comorbidities in Children With Congenital Heart Disease.

Outcomes for patients with neonatal heart disease are affected by numerous noncardiac and genetic factors. These can include neonatal concerns, such as prematurity and low birth weight, and congenital anomalies, such as airway, pulmonary, gastrointestinal, and genitourinary anomalies, and genetic syndromes. This section will serve as a summary of these issues and how they may affect the evaluation and management of a neonate with heart disease. These noncardiac factors are heavily influenced by conditions common to neonatologists, making a strong argument for multidisciplinary care with neonatologists, cardiologists, surgeons, anesthesiologists, and cardiovascular intensivists. Through this section and this project, we aim to facilitate a comprehensive approach to the care of neonates with congenital heart disease.

Cardiopulmonary Resuscitation in Infants and Children With Cardiac Disease: A Scientific Statement From the American Heart Association.

Cardiac arrest occurs at a higher rate in children with heart disease than in healthy children. Pediatric basic life support and advanced life support guidelines focus on delivering high-quality resuscitation in children with normal hearts. The complexity and variability in pediatric heart disease pose unique challenges during resuscitation. A writing group appointed by the American Heart Association reviewed the literature addressing resuscitation in children with heart disease. MEDLINE and Google Scholar databases were searched from 1966 to 2015, cross-referencing pediatric heart disease with pertinent resuscitation search terms. The American College of Cardiology/American Heart Association classification of recommendations and levels of evidence for practice guidelines were used. The recommendations in this statement concur with the critical components of the 2015 American Heart Association pediatric basic life support and pediatric advanced life support guidelines and are meant to serve as a resuscitation supplement. This statement is meant for caregivers of children with heart disease in the prehospital and in-hospital settings. Understanding the anatomy and physiology of the high-risk pediatric cardiac population will promote early recognition and treatment of decompensation to prevent cardiac arrest, increase survival from cardiac arrest by providing high-quality resuscitations, and improve outcomes with postresuscitation care.

Preoperative trophic feeds in neonates with hypoplastic left heart syndrome.

OBJECTIVE: The aim of this is study is to determine if preoperative trophic feeds (TFs) can improve outcomes after Norwood palliation. DESIGN: This is a retrospective cohort study. SETTING: The setting is a pediatric cardiovascular intensive care unit in a tertiary hospital. PATIENTS: The patients were 50 consecutive patients with hypoplastic left heart, excluding 5 patients with prematurity or other significant comorbidities. Thirty-one patients that received preoperative TFs (20-30 mL/kg) were compared with 14 that remained nothing by mouth. Decision to initiate feeds was based on attending preference. All patients had protocolized feeds postoperatively, with initiation via transpyloric tube when patient was on minimal inotropes. OUTCOME MEASURES: Demographic, feeding, and other clinical outcome data were collected retrospectively from the patient medical record. RESULTS: There were no differences in demographics or preoperative risk factors (mechanical ventilation and lowest pH) between the two groups. Overall survival to discharge was 78% (25/31 TF, 10/14 nothing by mouth, P = .7). Neonates receiving TFs had less fluid administration in the cardiovascular operating room (P = .002), a more negative 48-hour postoperative fluid balance (P = .03), and median 3 days shorter duration of mechanical ventilation (P = .006). Trophic feeds patients also had a nonsignificant trend toward lower peak lactic acid (P = .06), lower inotropic score (P = .15), shorter hospital length of stay (P = .19), and faster time to tolerance of full enteral and oral feeds by 3 and 8 days, respectively (P = .06 and .01). There were no episodes of necrotizing enterocolitis in either group. CONCLUSIONS: Preoperative TFs before Norwood palliation appear safe and are associated with shorter duration of mechanical ventilation, a trend toward more stable postoperative hemodynamics, less fluid overload, and earlier postoperative feeding tolerance.

Ultrasound-guided femoral vein catheterization in neonates with cardiac disease*.

OBJECTIVE: To describe a novel technique for real-time, ultrasound-guided femoral vein catheterization in neonates with cardiac disease, and to compare it to a contemporaneous cohort of neonates undergoing femoral vein central venous line placement via landmark technique. DESIGN: Retrospective cohort study of data extracted from a quality improvement database. SETTING: Pediatric cardiac intensive care unit and cardiovascular operating room in pediatric tertiary hospital. PATIENTS: One hundred fifteen neonates (mean weight, 3.07 ± 0.41 kg) with cardiac disease who underwent femoral central venous line attempts from January 2009 to September 2011. MEASUREMENTS AND MAIN RESULTS: Study populations were similar in age, weight, and Risk Adjustment for Congenital Heart Surgery-1 category, but differed in intubation status (32% vs. 100%, ultrasound vs. landmark, p < .0001). Central venous line success rate was superior in the ultrasound group: 72 of the 76 (94.7%) vs. 31 of the 39 (79.5%), p = .02. Ultrasound group also had a superior first (75% vs. 30.8 %) and second attempt success rate (90.8% vs. 51.3%), p value for both < .0001. Inadvertent arterial puncture occurred less frequently in the ultrasound group: four of the 76 (5.3%) vs. nine of the 39 (23.1%), p = .01. There was a trend toward more venous thrombosis in the landmark group, 16 of the 39 (41%) vs. 18 of the 76 (23.7%), p = .08. Among all 115 subjects, there was a very strong association between greater than two central venous line attempts and the odds of being diagnosed with a deep venous thrombosis (odds ratio, 9.3; 95% confidence interval 3.5-24.8) and the odds of suffering an inadvertent femoral arterial puncture during the central venous line event (odds ratio, 8.8; 95% confidence interval 10.6-730). CONCLUSIONS: This novel long-axis real-time ultrasound technique facilitates placement of femoral vein central venous line in critically ill neonates with cardiac disease at a higher rate of success with fewer attempts and lower occurrence of complications when compared with the landmark technique.

Early initiation of arginine vasopressin infusion in neonates after complex cardiac surgery.

OBJECTIVE: To describe our experience with low-dose arginine vasopressin infusions (0.0003 U/kg/min) initiated in the operating room after the Norwood procedure or arterial switch operation. DESIGN: Retrospective cohort study of 37 consecutive neonates. SETTING: Pediatric cardiovascular intensive care unit in a tertiary hospital. SUBJECTS: Nineteen patients that received low-dose arginine vasopressin infusion instituted in the operating room (arginine vasopressin+) were compared to 18 patients that did not receive early arginine vasopressin infusion (arginine vasopressin-). INTERVENTIONS: None. RESULTS: When comparing arginine vasopressin+ and arginine vasopressin- in the first 24 hrs after cardiovascular intensive care unit admission, there was no difference in demographic variables, heart rate, blood pressure, central venous pressure, maximum lactate, maximum arterial and central venous saturation difference, urine output, chest tube output, or peritoneal drain output. Mean fluid resuscitation in the first 24 hrs was significantly lower in the arginine vasopressin+ group compared to the arginine vasopressin- group (182 ± 61 mL/kg vs. 223 ± 53 mL/kg, p = .03). The arginine vasopressin+ group also reached median net negative cumulative fluid balance sooner (55 hrs: interquartile range 45, 74 vs. 76 hrs: interquartile range 69, 92; p = .02). Median maximum inotrope score in the first 24 hrs was significantly lower in arginine vasopressin+ (9: interquartile range 5, 12.5 vs. 16.5: interquartile range 10.3, 22.1; p = .02). There was a nonsignificant trend toward shorter duration of mechanical ventilation and cardiovascular intensive care unit length of stay in the arginine vasopressin+ group. The lowest serum sodium in the first 48 hrs was significantly lower in arginine vasopressin+ (132 vs. 137 mmol/L, p = .01). CONCLUSION: Low-dose arginine vasopressin infusion initiated in the operating room after complex neonatal cardiac surgery was associated with decreased fluid resuscitation and catecholamine requirements in the first 24 postoperative hours.

The proapoptotic BH3-only, Bcl-2 family member, Puma is critical for acute ethanol-induced neuronal apoptosis.

Synaptogenesis in humans occurs in the last trimester of gestation and in the first few years of life, whereas it occurs in the postnatal period in rodents. A single exposure of neonatal rodents to ethanol during this period evokes extensive neuronal apoptosis. Previous studies indicate that ethanol triggers the intrinsic apoptotic pathway in neurons, and that this requires the multi-BH domain, proapoptotic Bcl-2 family member Bax. To define the upstream regulators of this apoptotic pathway, we examined the possible roles of p53 and a subclass of proapoptotic Bcl-2 family members (i.e. the BH3 domain-only proteins) in neonatal wild-type and gene-targeted mice that lack these cell death inducers. Acute ethanol exposure produced greater caspase-3 activation and neuronal apoptosis in wild-type mice than in saline-treated littermate controls. Loss of p53-upregulated mediator of apoptosis (Puma) resulted in marked protection from ethanol-induced caspase-3 activation and apoptosis. Although Puma expression has been reported to be regulated by p53, p53-deficient mice exhibited a similar extent of ethanol-induced caspase-3 activation and neuronal apoptosis as wild-type mice. Mice deficient in other proapoptotic BH3-only proteins, including Noxa, Bim, or Hrk, showed no significant protection from ethanol-induced neuronal apoptosis. Collectively, these studies indicate a p53-independent, Bax- and Puma-dependent mechanism of neuronal apoptosis and identify Puma as a possible molecular target for inhibiting the effects of intrauterine ethanol exposure in humans.