Longitudinal Assessment of Growth in Hypoplastic Left Heart Syndrome: Results From the Single Ventricle Reconstruction Trial

Background: We sought to characterize growth between birth and age 3 years in infants with hypoplastic left heart syndrome who underwent the Norwood procedure. Methods and Results: We performed a secondary analysis using the Single Ventricle Reconstruction Trial database after excluding patients 2 SD below normal). Failure to find consistent risk factors supports the strategy of tailoring nutritional therapies to patient‐ and stage‐specific targets. Clinical Trial Registration URL: http://clinicaltrials.gov/. Unique identifier: NCT00115934

Intensive Care Unit and Acute Care Unit Length of Stay After Congenital Heart Surgery

BackgroundPostoperative length of stay (LOS) is an important quality metric and is known to vary widely across hospitals after congenital heart surgery. Whether this variability is explained by factors associated with the intensive care unit (ICU) or acute care unit (ACU) remains unclear. We evaluated the relationship between ICU and ACU LOS and the impact of ACU characteristics on postoperative LOS.MethodsHospitalizations for congenital heart surgery within the Pediatric Cardiac Critical Care Consortium (PC4) registry (August 2014 to February 2018) were included. Models were developed for ICU, ACU, and postoperative LOS by adjusting for differences in case-mix across hospitals. PC4 hospitals participating in the Pediatric Acute Care Cardiology Collaborative (PAC3) were also surveyed on ACU organizational factors and practice patterns.ResultsOverall, 19,674 hospitalizations across 27 hospitals were included. There was significant variation in ICU and ACU LOS. Postperative LOS appeared to be most closely related to ICU LOS; 75% (6 of 8) of hospitals with shorter than expected postoperative LOS also had shorter than expected ICU LOS. A clear relationship between postoperative and ACU LOS was not observed. Hospitals with an ACU able to provide higher-acuity care as indexed according to the PAC3 survey were more likely to have shorter postoperative LOS (P < .01).ConclusionsFor hospitals that achieve shorter than expected postoperative LOS after congenital heart surgery, ICU LOS appears to be the primary driver. Higher-acuity resources in the ACU may be an important factor facilitating earlier transfer from the ICU. These data are key to informing quality improvement initiatives geared toward reducing postoperative LOS

Successful Medical-Surgical Management of Intracardiac Thrombosis and Pulmonary Pseudoaneurysms in an Adolescent With Hughes-Stovin Syndrome

We present an adolescent male with a single intracardiac mass and pulmonary emboli, complicated by peripheral venous thrombosis and subsequent development of pulmonary pseudoaneurysms, leading to diagnosis of Hughes-Stovin syndrome. Remission was achieved with cyclophosphamide, corticosteroids, and pseudoaneurysm resection and maintained with infliximab and methotrexate

Variation in Care Practices Across Pediatric Acute Care Cardiology Units: Results of the Pediatric Acute Care Cardiology Collaborative (PAC

BackgroundThe Pediatric Acute Care Cardiology Collaborative (PAC3) was established in 2014 to improve the quality, value, and experience of hospitalâ based cardiac acute care outside of the intensive care unit. An initial PAC3 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.MethodsA 126â 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).ResultsSurveys 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 lesionâ specific postoperative pathways and approximately twoâ thirds had protocols for singleâ ventricle patients.ConclusionsThe PAC3 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.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/149524/1/chd12739.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/149524/2/chd12739_am.pd

Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial

ObjectiveFor infants with single ventricle malformations undergoing staged repair, interstage mortality is reported at 2% to 20%. The Single Ventricle Reconstruction trial randomized subjects with a single morphologic right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt (MBTS) or a right ventricle-to-pulmonary artery shunt (RVPAS). The aim of this analysis was to explore the associations of interstage mortality and shunt type, and demographic, anatomic, and perioperative factors.MethodsParticipants in the Single Ventricle Reconstruction trial who survived to discharge after the Norwood procedure were included (n = 426). Interstage mortality was defined as death postdischarge after the Norwood procedure and before the stage II procedure. Univariate analysis and multivariable logistic regression were performed adjusting for site.ResultsOverall interstage mortality was 50 of 426 (12%)-13 of 225 (6%) for RVPAS and 37 of 201 (18%) for MBTS (odds ratio [OR] for MBTS, 3.4; P < .001). When moderate to severe postoperative atrioventricular valve regurgitation (AVVR) was present, interstage mortality was similar between shunt types. Interstage mortality was independently associated with gestational age less than 37 weeks (OR, 3.9; P = .008), Hispanic ethnicity (OR, 2.6; P = .04), aortic atresia/mitral atresia (OR, 2.3; P = .03), greater number of post-Norwood complications (OR, 1.2; P = .006), census block poverty level (P = .003), and MBTS in subjects with no or mild postoperative AVVR (OR, 9.7; P < .001).ConclusionsInterstage mortality remains high at 12% and is increased with the MBTS compared with the RVPAS if postoperative AVVR is absent or mild. Preterm delivery, anatomic, and socioeconomic factors are also important. Avoiding preterm delivery when possible and close surveillance after Norwood hospitalization for infants with identified risk factors may reduce interstage mortality