Long QT syndrome with a de novo CALM2 mutation in a 4-year-old boy.

BACKGROUND: Human calmodulin (CALM) gene mutation has been reported to be related to inherited arrhythmia syndromes, but the genotype-phenotype relationship remains unclear. METHODS AND RESULTS: We report here a 4-year-old boy who had cardiac arrest while playing in a kindergarten playground. Cardiopulmonary resuscitation was initiated immediately. Eleven minutes after the cardiac arrest, ambulance crews arrived and an automated external defibrillator was attached. His heart rhythm, which was ventricular fibrillation (VF), was returned to sinus rhythm after only one shock delivery. The boy was brought to hospital by air ambulance. During transfer, electrocardiogram (ECG) showed transient VF. On arrival, chest radiograph showed a cardiothoracic ratio of 55% without pulmonary congestion. A 12-lead ECG showed a normal sinus rhythm, biphasic T wave, and prolongation of the corrected QT interval. On ECG, VF was preceded by torsade de pointes or frequent polymorphic premature ventricular contractions (PVC). Echocardiography showed a normal heart structure with decreased cardiac function. On the second day of hospitalization, ECG showed remarkable QT prolongation, T-wave alternans, and frequent PVC. Thereafter, propranolol was started. The ECG showed rapid improvement of QT prolongation and T-wave abnormality. Genetic test indicated a CALM2 mutation, and he was diagnosed with long QT syndrome-15 (LQT15). CONCLUSIONS: CALM mutations cause long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT) and idiopathic VF. This patient with a CALM2 p.N98S mutation had both phenotypes of LQTS and CPVT.

Effect of Advanced Airway Management by Paramedics During Out-of-Hospital Cardiac Arrest on Chest Compression Fraction and Return of Spontaneous Circulation.

PURPOSE: To obtain effective systemic blood flow and coronary perfusion by chest compressions during cardiopulmonary resuscitation, it is recommended that the interruption time of chest compressions be kept to a minimum, and that the chest compression fraction (CCF) should be kept high. In this study, we examined the effects of advanced airway management by paramedics in out-of-hospital cardiac arrest (OHCA) cases on CCF and on return of spontaneous circulation (ROSC) before arrival at the hospital. PARTICIPANTS AND METHODS: A total of 283 adult, non-traumatic OHCA cases who were in cardiopulmonary arrest at the time of EMS contact between April 2015 and March 2017 were registered for this study. We retrospectively investigated the presence or absence of advanced airway clearance, CCF and ROSC during CPR. CCF was calculated by measuring the chest compression interruption time from the ECG waveform recorded on a semiautomatic defibrillator (Philips HeartStart MRX). The data obtained were recorded on a computer, and comparisons between groups were examined using an untested t-test and χ2- test. RESULTS: Of the 283 patients with OHCA, 159 were included in the analysis. The CCF of the AAM group was 89.4%, which was significantly higher than that in the BMV group (84.3%) (P<0.01). Forty-one patients had ROSC at the time of arrival at the hospital, and the CCF of ROSC patients was 89.7%, which was significantly higher than that of non-ROSC patients (87.2%) (P<0.01). ROSC was also obtained in 31.8% of the patients in the AAM group, which was significantly higher than that in the BMV group (12.2%). CONCLUSION: In this study, we found that advanced airway management during prehospital emergency transport by paramedics showed high CCF and ROSC rates and contributed to improving the prognosis of OHCA patients through high-quality resuscitation.