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The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry captures information on interstage management of infants with hypoplastic left heart syndrome (HLHS). The purpose of this study was to identify interstage risk factors for increased resource use and adverse outcomes during bidirectional Glenn (BDG) hospitalization. All infants in the NPC-QIC registry (31 United States hospitals) undergoing BDG surgery were included (December 2009 to August 2010). Patient demographics, interstage variables, operative procedures, and complications were recorded. Days of hospitalization, ventilation, inotrope use, and complications were surrogates of resource use. Logistic regression analysis determined the associations between predictor variables and resource use. Of 162 infants, 105 (65 %) were males. At BDG, the median age was 155 days (range 78-128), mean weight-for-age z-score was -1.6 ± 1.1, mean length-for-age z-score was -1.5 ± 1.7, and mean preoperative oxygen saturation was 78 % ± 7 %. Caloric recommendations were met in 60 % of patients, and 85 % of patients participated in a home-surveillance program. Median days of intubation, inotrope use, and hospitalization were 1, 2, and 7, respectively. There were 4 post-BDG deaths and 55 complications. In multivariate analysis, lower weight-for-age z-score, female sex, and aortic atresia with mitral stenosis were associated with a higher risk of BDG complications. Meeting caloric recommendations before BDG was associated with fewer hospitalization days. Lower weight-for-age z-score was an independent and potentially modifiable risk factor for BDG complications. HLHS infants who met caloric recommendations before BDG had a lower duration of hospitalization at BDG. These data justify targeting nutrition in interstage strategies to improve outcomes and decrease costs for patients with HLHS.

Introduction.  Over the past three decades, significant advances in treatment have improved the mortality of children with cardiac disease. The effect of these advances on the prevalence of arterial ischemic stroke (AIS) is unknown. We describe AIS in children with cardiac disease in the modern era. Design.  The prospectively enrolled Intermountain Pediatric Stroke Database (including Utah, Wyoming, Idaho, and Nevada) was queried for all patients less than 18 years old with new‐onset AIS between January 1, 2003 and August 31, 2009. Medical records of patients with AIS and cardiac disease were reviewed for cardiac diagnosis, age at AIS, anticoagulant therapy, diuretics, hematocrit, bolus fluids, and ongoing morbidity. Data were analyzed using chi‐square test and a mixed‐effects Poisson regression growth curve model. Results.  AIS incidence in our catchment area was 0.01% (10.7/100 000; N = 97). The incidence of AIS in patients with cardiac disease was higher compared with AIS in the total population (incidence 0.13% [132/100 000], odds ratio [OR] 16.1, 95% confidence interval [CI; 9.7–25.9], P < 0.001). Of the 97 patients with AIS, 24 had cardiac disease (25%). The most common cardiac diagnosis was single ventricle (SV; 8/24, 33%). The incidence of AIS in patients with SV cardiac disease was higher compared with those with other cardiac diagnoses (incidence 1.38% [1380/100 000], OR 15.3, 95% CI [5.7–38.2], P < 0.001). Modeling the prevalence estimates reported since 1978, the prevalence of cardiac disease in AIS patients has remained unchanged across time (prevalence increase per each additional year, 0.5%, 95% CI [−2.1%, 3.1%], P= 0.71). Conclusion.  Children with cardiac disease (particularly those with SV) have increased risk for AIS. The prevalence is unchanged from reports over previous decades. AIS occurred in SV patients despite compliance with current anticoagulation recommendations. Future efforts should focus on best practices to prevent AIS in cardiac patients.

OBJECTIVESAfter completing this article, readers should be able to:. Describe where stenosis can occur. 2. Delineate the most likely cause of a murmur in the first week of life. 3. Describe the clinical course and treatment of aortic stenosis. 4. Describe the presentation of children who have pulmonary stenosis and the treatment of choice.

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