I(Kr) channel blockade to unmask occult congenital long QT syndrome.

BACKGROUND: Patients with genetic evidence of long QT syndromes type 1 and 2 (LQT1, associated with impaired outward potassium current I(Ks); and LQT2, associated with impaired outward potassium current I(Kr)) may have normal baseline QT intervals (phenotype/genotype discordance) and elude clinical detection. Beta-adrenergic stimulation may unmask occult LQT1, but no maneuver has consistently unmasked the LQT2 phenotype. OBJECTIVE: The purpose of this study was to test the repolarization reserve hypothesis (multiple challenges to repolarization are required to produce an abnormal phenotype), using subjects with LQT1 and LQT2 mutations but normal QT interval. We hypothesized that I(Kr) channel blockade would prolong the QT interval excessively in subjects with LQTS compared with controls and that I(Kr) channel blockade could unmask the abnormal LQTS phenotype in subjects with LQTS versus controls, as measured by the T peak-to-end interval (Tpe), a sensitive measure of abnormal repolarization. METHODS: Subjects with known LQT1 (n = 5) and LQT2 (n = 6) mutations but baseline QTc < or = 450 ms and age- and gender-matched controls (n = 22) received intravenous erythromycin (an I(Kr) blocker). RR, QRS, QT, and Tpe intervals were measured at baseline and after drug infusion. RESULTS: Erythromycin caused only modest QT prolongation in all groups. In contrast, Tpe was specifically prolonged by I(Kr) channel blockade in LQT2 subjects but not in LQT1 subjects or controls. CONCLUSION: Short-acting I(Kr) channel blockade, together with the sensitive repolarization measure Tpe, can unmask abnormal repolarization in LQT2. Our finding of abnormal repolarization in LQT2 subjects exposed to I(Kr) channel blockade supports the repolarization reserve hypothesis.

Rapid Development and Deployment of Ebola Readiness Training Across an Academic Health System: The Critical Role of Simulation Education, Consulting, and Systems Integration.

STATEMENT: In this article, we describe an Ebola preparedness initiative with implementation across an academic health system. Key stakeholder centers of various disciplines and clinical experts collaborated in the development and design. Subject matter experts in the areas of Centers for Disease Control and Prevention and World Health Organization protocols for personal protective equipment donning and doffing conducted initial train-the-trainer sessions for program instructors. These trainers represented a cross-section of key clinical responders and environmental services. Through a parallel development process, a blended learning curriculum consisting of online modules followed by on-site training sessions was developed and implemented in both the simulation laboratory and the actual clinical care spaces in preparation for a Department of Health inspection. Lessons learned included identification of the need for iterative refinement based on instructor and trainee feedback, the lack of tolerance of practitioners in wearing full-body personal protective equipment for extended periods, and the ability of a large system to mount a rapid response to a potential public health threat through leveraging of expertise of its Simulation Program, Center for Quality, Safety and Innovation as well as a wide variety of clinical departments.

Interventions to improve the timeliness of emergency care.

With a persistent trend of increasing emergency department (ED) volumes every year, services are intensifying. Thus, improving the timeliness of delivering emergency care should be a primary focus, both from an operational and from a research perspective. Much has been published on factors associated with delays in emergency care, and the next phase in this area of research will focus on exploring interventions to improve the timeliness of care. On June 1, 2011, Academic Emergency Medicine held a consensus conference titled "Interventions to Assure Quality in the Emergency Department." This article summarizes the findings of the breakout session that investigated interventions to improve the timeliness of emergency care. This article will explore the background on the concept of timeliness of emergency care, the current state of interventions that have been implemented to improve timeliness, and specific questions as a framework for a future research agenda.