Coronary Bypass in an Adolescent With Systemic Lupus Erythematosus and Acute Coronary Syndrome.

A 12-year-old female with active pediatric juvenile systemic lupus erythematosus presented to the emergency department because of episodes of oppressive central thoracic pain associated with pallor, sweating, and muscle weakness that persisted for >30 minutes. During the last episode, the electrocardiogram revealed alterations in cardiac repolarization coincident with progressive troponin T elevation. An angio computed tomography revealed a 20 mm long complete segmental obstruction of the proximal anterior descending artery that was confirmed by angiography. Because this extensive occlusion did not permit a noninvasive procedure, an off-pump coronary bypass of the internal mammary artery to the anterior descending artery was performed without complication. Six months after the procedure, myocardial function was good. To our knowledge, this is the first case report of an adolescent girl with acute coronary syndrome complicating juvenile systemic lupus erythematosus that was treated with a surgical procedure.

KCNH2 p.Gly262AlafsTer98: A New Threatening Variant Associated with Long QT Syndrome in a Spanish Cohort.

Long QT syndrome (LQTS) is an inherited (autosomal dominant) channelopathy associated with susceptibility to ventricular arrhythmias due to malfunction of ion channels in cardiomyocytes, that could lead to sudden death (SD). Most pathogenic variants are in the main 3 genes: KCNQ1 (LQT1), KCNH2 (LQT2) and SCN5A (LQT3). Efforts to improve the understanding of the genotype-phenotype relationship are essential to improve the medical clinical practice. In this study, we identified all index patients referred for NGS genetic sequencing due to LQTS, in a Spanish cohort, who were carriers of a new pathogenic variant (KCNH2 p.Gly262AlafsTer98). Genetic and clinical family screening was performed in order to describe its phenotypic characteristics. We identified 22 relatives of Romani ethnicity, who were carriers of the variant. Penetrance reached a 100% and adherence to medical treatment was low. There was a high rate of clinical events, particularly arrhythmic events and SD (1 in every 4 patients presented syncope, 1 presented an aborted SD, 2 obligated carriers suffered SD before the age of 40 and 4 out of 6 carriers of an implantable cardioverter-defibrillator (ICD) had appropriate ICD therapies. Correct adherence to medical treatment in all carriers should be specially encouraged in this population. ICD implantation decision in non-compliant patients, and refusing left cardiac sympathetic denervation, should be carefully outweighed.

[The checklists: A help or a hassle?] / Las listas de verificación: ¿una ayuda o una molestia?

Patient safety has become a central component of quality of care. One of the best known and most widely used security tool in all work settings is the checklist. The checklist is a tool that helps to not forget any step during the performance of a procedure, to do tasks with an established order, to control the fulfilment of a series of requirements or to collect data in a systematic way for its subsequent analysis. It is an aid to improve the efficiency of teamwork, promote communication, decrease variability, standardize care and improve patient safety. Main barriers to implementation are reviewed: staff attitudes, hierarchies, poor design, inadequate training, duplication with other work lists, work overload, cultural barriers, lack of replication or checklist closing time. Finally, its applications in Pediatrics are reviewed starting from the most widespread, the safety checklist of pediatric surgery, checklists in neonatal critical units, for safe delivery, for risk procedures, in pediatric intensive care and for pathology time-dependent emergent, e.g. pediatric trauma. It is necessary to highlight the role of leadership in the implantation of a checklist in any area of Pediatrics. There must be one or more people from the team with the support of the Heads of Service and Managers who lead the training of the personnel, direct the implementation of the LV, evaluate the results, inform the rest of the team and can modify the processes depending on the problems found.