Percutaneous Endoscopic Gastrostomy Enhances Interstage Growth in Infants With Hypoplastic Left Heart Syndrome.

OBJECTIVES: Infants with hypoplastic left heart syndrome undergoing staged palliation commonly experience chronic growth failure and malnutrition. Greater patient weight at stage 2 palliation (Glenn) is thought to be associated with improved perioperative outcomes. We aimed to compare weight for age z score and interstage growth velocity in children with and without a percutaneous endoscopic gastrostomy prior to Glenn and hypothesize that those with a percutaneous endoscopic gastrostomy experience-enhanced interstage growth and reduced malnutrition rates. DESIGN: Single-center, retrospective cohort study. SETTING: A total of 259-bed, quaternary, pediatric referral center. PATIENTS: Infants with hypoplastic left heart syndrome from 2007 to 2016 with and without percutaneous endoscopic gastrostomy insertion after initial palliation (Norwood). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Primary outcomes were weight for age z score (at birth, Norwood, Norwood discharge, and Glenn), interstage growth velocity, and moderate-to-severe malnutrition (weight for age z score<-2) rates. Secondary outcomes were lengths of stay, mechanical ventilation rates after Glenn, and mortality. Statistical analyses included chi-square, Wilcoxon rank-sum, student's t, paired testing, and exploratory logistic regression. Of the 69 infants studied, 47 (68%) had percutaneous endoscopic gastrostomy insertion at a median of 156 (interquartile range, 115-158) days prior to Glenn. Among children with and without percutaneous endoscopic gastrostomy, we observed no differences in demographics, comorbidities, cardiothoracic surgical times, postoperative Glenn outcomes (length of stay, mechanical ventilation rate, peak 24-hr lactate, nitric oxide use, extracorporeal life support rate, or mortality), weight for age z score at birth, and weight for age z score at Norwood. At the time of percutaneous endoscopic gastrostomy insertion, weight for age z score was -2.5 ± 1.3 and subsequent growth velocity increased from 8 ± 7 to 40 ± 59 g/d (p < 0.01). From Norwood discharge to the date of Glenn, weight for age z score increased in infants with percutaneous endoscopic gastrostomy (-2.5 ± 1.1 to -1.5 ± 1.4 [p < 0.01]) with a large reduction in moderate-to-severe malnutrition rates (76-36%; p < 0.01). In general, weight for age z score at the time of Glenn was associated with reduced postoperative mortality (odds ratio, 0.3; 95% CI, 0.09-0.95; p = 0.04). CONCLUSIONS: Infants undergoing palliation for hypoplastic left heart syndrome with percutaneous endoscopic gastrostomy insertion prior to Glenn had improved growth velocity and dramatically reduced rates of moderate-to-severe malnutrition rates (40% reduction). In addition, we noted weight for age z score at when Glenn was associated with improved postoperative Glenn survival. No complications from percutaneous endoscopic gastrostomy were noted. Placement of a percutaneous endoscopic gastrostomy improved weight for age z score, enhanced interstage growth, and reduced malnutrition rates for this at-risk population of malnourished children.

Percutaneous Endoscopic Gastrostomy After Cardiothoracic Surgery in Children Less Than 2 Months Old: An Assessment of Long-Term Malnutrition Status and Gastrostomy Outcomes.

OBJECTIVES: Infants with critical congenital heart disease undergoing cardiothoracic surgery commonly experience chronic malnutrition and growth failure. We sought to determine whether placement of a percutaneous endoscopic gastrostomy was associated with reduced moderate-severe malnutrition status and to describe percutaneous endoscopic gastrostomy-related clinical and safety outcomes in this population. DESIGN: Single-center, retrospective cohort study. SETTING: Two hundred fifty-nine-bed, tertiary care, pediatric referral center. PATIENTS: Children with congenital heart disease less than 2 months old undergoing cardiothoracic surgery from 2007 to 2013 with and without percutaneous endoscopic gastrostomy. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Primary outcomes were weight for age z scores during hospitalization, at 6 months, and 1 year after cardiothoracic surgery. Secondary outcomes were frequency of percutaneous endoscopic gastrostomy revision, percutaneous endoscopic gastrostomy complications, and mortality. Statistical analyses included Wilcoxon rank-sum, Fisher exact, and Student t tests. Two hundred twenty-two subjects met study criteria, and 77 (35%) had percutaneous endoscopic gastrostomy placed at a mean of 45 ± 31 days after cardiothoracic surgery. No differences were noted for demographics, comorbidities, and weight for age z score at birth and at the time of cardiothoracic surgery. The percutaneous endoscopic gastrostomy cohort had greater Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery risk category (4 [4-5] vs 4 [2-4]) and length of stay (71 d [49-101 d] vs 26 d [15-42 d]). Mean weight for age z score at the time of percutaneous endoscopic gastrostomy was -2.8 ± 1.3. Frequency of moderate-severe malnutrition (weight for age z score, ≤ -2) was greater in children with percutaneous endoscopic gastrostomy at discharge (78% vs 48%), 6 months (61% vs 16%), and 1 year (41% vs 2%). Index mortality was lower in children with percutaneous endoscopic gastrostomy at 30 days (8% vs 0%) and hospital discharge (19% vs 4%). However, no mortality differences were observed after discharge. Growth velocity after percutaneous endoscopic gastrostomy was greater (44 ± 19 vs 10 ± 9 g/d). Children tolerated percutaneous endoscopic gastrostomy without hemodynamic compromise, minor percutaneous endoscopic gastrostomy complications, and anticipated percutaneous endoscopic gastrostomy revisions. Children without mortality had percutaneous endoscopic gastrostomy removal at a median duration of 253 days (133-545 d). Children with univentricular physiology had improved in-hospital mean growth velocity (6.3 vs 24.4 g/d; p < 0.01) and reduced 1-year rate moderate-severe malnutrition (66.7% vs 36.9%; p < 0.01) after percutaneous endoscopic gastrostomy placement. CONCLUSIONS: Percutaneous endoscopic gastrostomy placement was well tolerated and associated with improved postoperative growth velocity in children with critical congenital heart disease undergoing cardiothoracic surgery less than 2 months old. These findings were also noted in our subanalysis of children with univentricular physiology. Persistent rates of moderate-severe malnutrition were noted at 1-year follow-up. Although potential index mortality benefit was observed, definitive data are still needed.

Pulmonary atresia with intact ventricular septum and congenital left ventricular aneurysm.

Pulmonary atresia with intact ventricular septum and congenital left ventricular aneurysms are both rare, representing <1 and <0.1% of CHD, respectively. The association of left ventricular aneurysms and pulmonary atresia with intact ventricular septum has been documented in the literature, but all the previous case reports have involved children with acquired left ventricular aneurysms. We report an interesting case of a child with both pulmonary atresia with intact ventricular septum and a congenital left ventricular aneurysm. The combination of these two lesions ultimately affected the course of clinical care for the patient.

Postoperative Abdominal NIRS Values Predict Low Cardiac Output Syndrome in Neonates.

BACKGROUND: The development of low cardiac output syndrome (LCOS) after cardiopulmonary bypass (CPB) occurs in up to 25% of neonates and is associated with increased morbidity. Invasive cardiac output monitors such as pulmonary artery catheters have limited availability and are costly. Near-infrared spectroscopy (NIRS) is a noninvasive tool for monitoring regional oxygenation in neonates in the cardiac intensive care unit (CICU). We hypothesize that anterior abdominal NIRS may aid in the early identification of LCOS after cardiac surgery. METHODS: Prospective observational study from October 2013 to October 2014 of all neonates with congenital heart disease admitted to the CICU following CPB. Abdominal NIRS values were continuously recorded upon CICU admission and for the subsequent 24-hour period. The primary outcome was the development of LCOS. Low cardiac output syndrome was defined as the presence of metabolic lactic acidosis (pH < 7.3 and lactate > 4) or addition of a new vasoactive agent or a vasoactive inotropic score > 15. Autoregressive time series models were constructed for each patient based on the continuously recorded NIRS values, and patients were stratified by development of LCOS. RESULTS: Twenty-seven neonates met inclusion criteria, of whom 11 developed LCOS. Neonates who developed LCOS had lower constant NIRS values (49% vs. 66%, P < .001). Constant NIRS values less than 58% best predicted development of LCOS with a sensitivity of 100% and specificity of 69%. CONCLUSION: Lower constant anterior abdominal NIRS values in the early postoperative period may allow early identification of neonates at risk for LCOS.