Early and Midterm Outcomes in High-risk Single-ventricle Patients: Hybrid Vs Norwood Palliation.

BACKGROUND: Although overall outcomes have improved for single-ventricle patients, substantial morbidity and mortality remain for certain high-risk groups. The hybrid stage I procedure is an alternative to the Norwood operation for stage I palliation, but it remains unclear whether it is associated with improved outcomes in high-risk patients. METHODS: This single-center, nested, case-control study included high-risk patients with a systemic right ventricle who underwent hybrid stage I or Norwood palliation from January 2000 to December 2016. High-risk features included prematurity < 34 weeks, birth weight < 2.5 kg, restrictive/intact atrial septum, at least moderate atrioventricular valve regurgitation or right ventricular dysfunction, genetic or extracardiac anomalies, or left ventricular sinusoids. Patients were matched by presence of genetic anomaly, restrictive/intact atrial septum, and prematurity/weight < 2 kg. Early and midterm outcomes were compared in the matched hybrid vs Norwood groups. RESULTS: The study included 96 patients (35 hybrid, 61 Norwood). Despite improved 30-day survival in hybrid patients (91% vs 66%, P < .01), 1-year survival was similar between the hybrid and Norwood groups (46% vs 48%, P = .9). No hybrid patients required dialysis or extracorporeal membrane oxygenation after stage I palliation as compared with 19% and 22% of Norwood patients, respectively (both P < .01). Hybrid patients, however, required more unplanned reinterventions (43% vs 21%, P = .02). CONCLUSIONS: There remains significant morbidity and mortality among high-risk single-ventricle infants. Despite an early survival benefit, hybrid stage I palliation has not been associated with improved midterm outcomes at our center.

Pilot study analyzing automated ECG screening of hypertrophic cardiomyopathy.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is one of the leading causes of sudden cardiac death in athletes. However, preparticipation ECG screening has often been criticized for failing to meet cost-effectiveness thresholds, in part because of high false-positive rates and the cost of ECG screening itself. OBJECTIVE: The purpose of this study was to assess the testing characteristics of an automated ECG algorithm designed to screen for HCM in a multi-institutional pediatric cohort. METHODS: ECGs from patients with HCM aged 12 to 20 years from 3 pediatric institutions were screened for ECG criteria for HCM using a previously described automated computer algorithm developed specifically for HCM ECG screening. The results were compared to a known healthy pediatric cohort. The studies then were read by trained electrophysiologists using standard ECG criteria and compared to the results of automated screening. RESULTS: One hundred twenty-eight ECGs from unique patients with phenotypic HCM were obtained and compared with 256 studies from healthy control patients matched in 2:1 fashion. When presented with the ECGs, the non-voltage-based algorithm resulted in 81.2% sensitivity and 90.7% specificity. A trained electrophysiologist read the same data according to the Seattle Criteria, with 71% sensitivity with 95.7% specificity. The sensitivity of screening as well as the components of the ECG screening itself varied by institution. CONCLUSION: This pilot study demonstrates a potential for automated ECG screening algorithms to detect HCM with testing characteristics similar to that of a trained electrophysiologist. In addition, there appear to be differences in ECG characteristics between patient populations, which may account for the difficulties in universal screening.