Variation in care for children undergoing the Fontan operation for hypoplastic left heart syndrome.

BACKGROUND: The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion. METHODS: Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7 days). RESULTS: The median Fontan age (14 centres, 320 patients) was 3.1 years (range from 1.7 to 3.9), and the weight-for-age z-score was -0.56 (-1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4-100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3-100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7-33%). The length of stay was 9.5 days (9-12); 15% (6-33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98). CONCLUSIONS: Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.

Speckle-Tracking Echocardiography Improves Pre-operative Risk Stratification Before the Total Cavopulmonary Connection.

INTRODUCTION: Single-ventricle patients with elevated pulmonary vascular resistance (PVR) or end-diastolic pressure (EDP) are excluded from undergoing total cavopulmonary connection (TCPC). However, a subset of patients deemed to be at acceptable risk experience prolonged length of stay (LOS) after TCPC. Routine assessment of ventricular function has been inadequate in identifying these high-risk patients. Speckle-tracking echocardiography (STE) is a novel method for assessment of myocardial deformation that may be useful in single-ventricle patients. The aim of this study was to perform a contemporary preoperative risk assessment for prolonged LOS to determine whether STE improves risk stratification before TCPC. METHODS: Our single institution's perioperative data were retrospectively collected. The primary outcome was postoperative LOS >14 days. Longitudinal and circumferential STE deformation measures were analyzed on echocardiograms obtained during preoperative catheterization. Patient-specific, echocardiographic, and catheterization data were included in multivariable logistic regression. Receiver operating characteristic area under the curves (AUC) were analyzed. RESULTS: From 2007 to 2014, 135 patients who underwent TCPC were included in the analysis. The median LOS was 11 (IQR 9-14) days. The PVR (P < .01) and circumferential strain rate (CSR) (P < .01) were the only variables independently associated with LOS >14 days. For every 0.1 s-1 CSR increased, there was a 20% increased odds of prolonged LOS. The AUC for CSR was 0.70. The AUC for PVR and EDP combined was 0.68. The AUC for PVR, EDP, and CSR combined was 0.73. CONCLUSION: Preoperative CSR is independently associated with LOS >14 days and improves preoperative risk stratification in patients undergoing TCPC.

ECG-synchronized CT angiography in 324 consecutive pediatric patients: spectrum of indications and trends in radiation dose.

The aim of the study is to describe the spectrum of indications for pediatric ECG-synchronized CT angiography (CTA), the main determinants of radiation exposure, and trends in radiation dose over time at a single, tertiary referral center. The study was IRB approved and HIPAA compliant with informed consent waived. Between 2005 and 2013, 324 pediatric patients underwent ECG-synchronized CTA to evaluate known or suspected cardiovascular abnormalities (109 female, median age 8.1 years). The effective dose (ED) was calculated using age-specific correction factors. Univariate and multivariate regression analyses were performed to identify predictors of radiation dose. The most common primary indications for the CTA examinations included known or suspected coronary pathologies (n = 166), complex congenital heart disease (n = 73), and aortic pathologies (n = 41). Median radiation exposure decreased from 12 mSv for patients examined in the years 2005-2007 to 1.2 mSv for patients examined in the years 2011-2013 (p < 0.001). Patients scanned using a tube potential of 80 kV (n = 259) had a significantly lower median radiation dose (1.4 mSv) compared to patients who were scanned at 100 kV (n = 46, median 6.3 mSv) or 120 kV (n = 19, median 19 mSv, p < 0.001). Tube voltage, followed by tube current and the method of ECG-synchronization were the strongest independent predictors of radiation dose. Growing experience with dose-saving techniques and CTA protocols tailored to the pediatric population have led to a tenfold reduction in radiation dose over recent years and now allow routinely performing ECG-synchronized CTA in children with a radiation dose on the order of 1 mSv.