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Background: The Pediatric Acute Care Cardiology Collaborative (PAC 3 ) was established in 2014 to improve the quality, value, and experience of hospital-based cardiac acute care outside of the intensive care unit. An initial PAC 3 project was a comprehensive survey to understand unit structure, practices, and resource utilization across the collaborative. This report aims to describe the previously unknown degree of practice variation across member institutions., Methods: A 126-stem question survey was developed with a total of 412 possible response fields across nine domains including demographics, staffing, available resources and therapies, and standard care practices. Five supplemental questions addressed surgical case volume and number of cardiac acute care unit (CACU) admissions. Responses were recorded and stored in Research Electronic Data Capture (REDCap)., Results: Surveys were completed by 31 out of 34 centers (91%) with minimal incomplete fields. A majority (61%) of centers have a single dedicated CACU, which is contiguous or adjacent to the intensive care unit in 48%. A nurse staffing ratio of 3:1 is most common (71%) and most (84%) centers employed a resource nurse. Centralized wireless rhythm monitoring is used in 84% of centers with 54% staffed continuously. There was significant variation in the use of noninvasive respiratory support, vasoactive infusions, and ventricular assist devices across the collaborative. Approximately half of the surveyed centers had lesion-specific postoperative pathways and approximately two-thirds had protocols for single-ventricle patients., Conclusions: The PAC 3 hospital survey is the most comprehensive description of systems and care practices unique to CACUs to date. There exists considerable heterogeneity among unit composition and variation in care practices. These variations may allow for identification of best practices and improved quality of care for patients., (© 2019 Wiley Periodicals, Inc.)

Objective: To determine whether infundibular morphology is associated with coronary ostial atresia and/or right ventricle dependent coronary circulation in patients with pulmonary atresia with intact ventricular septum., Design: Neonatal echocardiograms of patients with pulmonary atresia with intact ventricular septum were evaluated for tricuspid valve size and infundibular anatomy (muscular pulmonary atresia vs. membranous pulmonary atresia). Right ventricle dependent coronary circulation and coronary ostial atresia were diagnosed angiographically. Medical record review determined patient outcome., Setting: Tertiary-care pediatric hospital., Results: Of 72 patients, 24 had muscular pulmonary atresia including 23 with right ventricle dependent coronary circulation. Fourteen of these had unilateral or bilateral coronary ostial atresia. Of 46 patients with membranous pulmonary atresia, 5 five had right ventricle dependent coronary circulation and none had coronary ostial atresia. Muscular pulmonary atresia patients had smaller tricuspid valve z-scores and were less likely to achieve a biventricular repair than those with membranous pulmonary atresia (P < .01). Muscular pulmonary atresia had 82% sensitivity, 98% specificity, and 96% positive predictive value for presence of right ventricle dependent coronary circulation. In the group with membranous pulmonary atresia, there were two deaths, no transplants, and 23 (48%) achieved a biventricular repair. In contrast, of the 24 with muscular pulmonary atresia, there were seven deaths, two transplants, and no biventricular repairs., Conclusions: In this cohort, muscular pulmonary atresia was strongly associated with right ventricle dependent coronary circulation and coronary ostial atresia, and appears to be a useful morphologic marker for poor outcome among pulmonary atresia with intact ventricular septum patients. This information may be useful during prenatal counseling and for presurgical evaluation., (© 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.)

Objectives: The objectives of this study were to characterize the exercise function of patients treated with balloon aortic valvuloplasty at 6 years old with a history of neonatal AS to undergo exercise testing., Results: We enrolled 30 patients (median age 13.1 years) who underwent balloon aortic valvuloplasty at a median age of 12 days. At time of exercise testing, the median maximum Doppler AS gradient was 34 mm Hg (0-70 mm Hg); 11 patients had moderate or severe aortic regurgitation. All patients were asymptomatic. Overall, peak oxygen consumption (VO(2)) was below normal (87 +/- 18% predicted; P < .001), and was severely depressed (