Specificity and sensitivity of automated external defibrillator rhythm analysis in infants and children.

STUDY OBJECTIVES: The rhythm detection algorithms of automated external defibrillators have been derived from adult rhythms, and their ability to discriminate between shockable and nonshockable rhythms in children is largely unknown. This study evaluates the performance of 1 automated external defibrillator algorithm in infants and children and evaluates algorithm performance with anterior-posterior versus sternal-apex lead placement. METHODS: We enrolled pediatric patients in a critical care unit, an electrophysiology laboratory, and a cardiac operating room. A monitor-defibrillator recorded ECGs by means of standard defibrillation-monitor pads. Selected 15-second rhythm samples were played into a LIFEPAK 500 automated external defibrillator, and the automated external defibrillator "shock/no shock" decision was documented. To determine sensitivity and specificity, the automated external defibrillator decision was compared with the "shockable" versus "nonshockable" rhythm classification provided by 3 expert clinicians who were blinded to the automated external defibrillator decision. RESULTS: We recorded 1,561 rhythm samples from 203 pediatric patients (median age 11 months; range, day of birth to 7 years). The automated external defibrillator recommended a shock for 72 of 73 rhythm samples classified as coarse ventricular fibrillation by expert review (sensitivity 99%; 95% confidence interval [CI] 93% to 100%); and correctly reached a "no shock advised" decision for 1,465 of 1,472 rhythm samples classified as nonshockable by experts (specificity 99.5%). Specificity was 99.1% (95% CI 97.8% to 99.8%) with the sternal-apex lead and 99.4% (95% CI 98.1% to 99.9%) with the anterior-posterior lead. CONCLUSION: This automated external defibrillator algorithm has high specificity and sensitivity when used in infants and children with either sternal-apex or anterior-posterior lead placement.

Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis.

Primary sclerosing cholangitis (PSC) and cystic fibrosis (CF) are both slowly progressive cholestatic liver diseases characterized by fibro-obliterative inflammation of the biliary tract. We hypothesized that dysfunction of the CF gene product, cystic fibrosis transmembrane conductance regulator (CFTR), may explain why a subset of patients with inflammatory bowel disease develop PSC. We prospectively evaluated CFTR genotype and phenotype in patients with PSC ( n=19) compared with patients with inflammatory bowel disease and no liver disease ( n=18), primary biliary cirrhosis ( n=17), CF ( n=81), and healthy controls ( n=51). Genetic analysis of the CFTR gene in PSC patients compared with disease controls (primary biliary cirrhosis and inflammatory bowel disease) demonstrated a significantly increased number of mutations/variants in the PSC group (37% vs 8.6% of disease controls, P=0.02). None of the PSC patients carried two mutations/variants. Of PSC patients, 89% carried the 1540G-variant-containing genotypes (resulting in decreased functional CFTR) compared with 57% of disease controls ( P=0.03). Only one of 19 PSC patients had neither a CFTR mutation nor the 1540G variant. CFTR chloride channel function assessed by nasal potential difference testing demonstrated a reduced median isoproterenol response of 14 mV in PSC patients compared with 19 mV in disease controls ( P=0.04) and 21 mV in healthy controls ( P=0.003). These data indicate that there is an increased prevalence of CFTR abnormalities in PSC as demonstrated by molecular and functional analyses and that these abnormalities may contribute to the development of PSC in a subset of patients with inflammatory bowel disease.