Utility and acceptability of remote 6-lead electrocardiographic monitoring in children with inherited cardiac conditions.

OBJECTIVE: This pilot study sought to investigate the utility and acceptability of the KardiaMobile 6-lead ECG (KM6LECG) as a tool for remote monitoring in children with inherited cardiac conditions. DESIGN: A single-centre prospective cohort study. Children underwent standard clinical evaluation including a 12-lead ECG and a KM6LECG in the clinic. Participants recorded KM6LECGs monthly at home for 3 months. Families completed a questionnaire on their experience. SETTING: Great Ormond Street Hospital Centre for Inherited Cardiovascular Diseases. PARTICIPANTS: 64 children: 22 with hypertrophic cardiomyopathy (HCM); 22 with long QT syndrome and 20 unaffected siblings (controls). MAIN OUTCOME MEASURES: Comparison of data extracted from the clinic 12-lead ECG and supervised KM6LECG, and the supervised and unsupervised KM6LECG recording. RESULTS: Of 64 children (35% female, mean age 12 years), 58 had a baseline 12-lead ECG and appropriate baseline KM6LECG. In children with HCM, abnormalities in ventricular depolarisation/repolarisation in the limb leads of the 12-lead ECG were reliably reproduced. From the whole cohort, there was a strong positive correlation between the corrected QT interval from the 12-lead ECG and baseline KM6LECG (intraclass correlation coefficient=0.839) and baseline KM6LECG with an unsupervised KM6LECG (intraclass correlation coefficient=0.736). Suspected 'lead' misplacement impacted 18% of unsupervised recordings. Overall, the acceptability of the KM6LECG to families was good. CONCLUSIONS: The KM6LECG provides an accurate tool for assessing some ECG abnormalities associated with paediatric inherited cardiovascular disease and may provide a useful at-home adjunct to face-to-face clinical care of children requiring ECG assessment.

Cardiopulmonary capacity is reduced in children with ventricular arrhythmia.

BACKGROUND: Premature ventricular contractions (PVCs) are frequently seen in children and are considered benign. A substantial group of adolescents with PVCs complain about a broad range of clinical symptoms, including low exertion tolerance. OBJECTIVE: The purpose of this study was to evaluate prospectively whether ventricular arrhythmia affects physical performance in adolescents with normal left ventricular function, using a cardiopulmonary exercise test (CPET) and evaluating the electrocardiographic (ECG) characteristics of patients with PVCs with regard to exercise capacity. METHODS: The study group consisted of 49 children with PVCs and normal left ventricular function. The control group consisted of 36 healthy volunteers. Standard ECG, 24-hour Holter ECG, and CPET were performed. PVCs were analyzed for QRS duration, bundle branch block pattern, QRS axis, and coupling interval (CInt). For CPET, heart rate (HR), oxygen uptake (VO2max), predicted VO2max, and VO2max expressed as a percentage of the predicted value (%VO2) were measured. RESULTS: In 37 patients (76%), arrhythmia subsided during exercise. Patients achieved lower VO2max (32.9 ± 6.3 mL/min/kg) than controls (40.4 ± 6.7 mL/min/kg; P <.01). %VO2 was 71.0 ± 13.7 in patients and 79.3 ± 12.2 in controls (P <.01). Exercise HR at which PVCs subsided correlated with VO2max (r = 0.3; P = .07). Patients with persisting arrhythmia performed worse than those in whom arrhythmia subsided during exercise (VO2max, P <.01; %VO2,P <.01). No correlation between QRS and CInt parameters and VO2max was observed. CONCLUSION: Patients with PVCs have lower aerobic capacity than their healthy peers. Further worsening of exercise capacity is present when PVCs are preserved during effort.

Electrocardiographic Parameters Associated with Adverse Outcomes in Children with Cardiomyopathies.

Cardiomyopathies have a low prevalence in children and thus may lead to malignant ventricular arrhythmias or the progression of heart failure, resulting in death. In adults, the QRS-T angle derived from ECG has been associated with adverse outcomes in patients with hypertrophic and dilated cardiomyopathies. We aimed to assess the electrocardiographic parameters, including QRS-T angle, associated with adverse cardiac events in children with cardiomyopathies. Forty-two children with cardiomyopathies were included in this study: 19 with dilated cardiomyopathy, 17 with hypertrophic cardiomyopathy, and 6 with left ventricular non-compaction. Additionally, 19 control subjects were recruited. In terms of ECG parameters, the QRS-T angle was significantly greater among patients with adverse outcomes compared to patients without the end points of the study (133° vs. 65°, p < 0.001). On Kaplan−Meier survival curves, QRS-T angle > 120°, increased serum concentrations of NT-proBNP and troponin I levels as well as greater NYHA or Ross scale were associated with the greatest risk of unfavorable outcome. The QRS-T angle appears to be a valuable component of 12-lead ECG interpretation, and might be helpful in outlining patients with the greatest cardiovascular risk. Additionally, serum biomarkers such as NT-proBNP (p = 0.003) and troponin (p < 0.001) are useful in outlining patients with the worst survival.

Left Ventricular Noncompaction-A Systematic Review of Risk Factors in the Pediatric Population.

Left ventricular noncompaction (LVNC) is a heterogeneous, often hereditary group of diseases, which may have diverse clinical manifestations. This article reviews the risk factors for unfavorable outcomes of LVNC in children, as well as discuss the diagnostic methods and the differences between pediatric and adult LVNC. Through a systematic review of the literature, a total of 1983 articles were outlined; 23 of them met the inclusion criteria. In echocardiography the following have been associated with adverse outcomes in children: Left ventricular ejection fraction, end-diastolic dimension, left ventricular posterior wall compaction, and decreased strains. T-wave abnormalities and increased spatial peak QRS-T angle in ECG, as well as arrhythmia, were observed in children at greater risk. Cardiac magnetic resonance is a valuable tool to identify those with systolic dysfunction and late gadolinium enhancement. Genetic testing appears to help identify children at risk, because mutations in particular genes have been associated with worse outcomes. ECG and imaging tests, such as echocardiography and magnetic resonance, help outline risk factors for unfavorable outcomes of LVNC in children and in identifying outpatients who require more attention. Refining the current diagnostic criteria is crucial to avoid inadequate restrain from physical activity.

Impact of a Single Session of Inhalation Technique Training on Inhalation Skills and the Course of Asthma and COPD.

BACKGROUND: A significant percentage of patients with asthma and COPD do not use their inhalers properly. The aim of this study was to evaluate the impact of a single session of inhalation technique training on the frequency of inhalation errors and the course of asthma and COPD. METHODS: This randomized controlled trial included adults who had been diagnosed with and treated for asthma or COPD with at least one inhaler use daily on a regular basis. All subjects were followed for 6 months, at which time their inhalation technique was assessed and an individual inhalation training (study group) or a sham procedure (control group) was applied. The number of mishandlings was calculated as the ratio of the number of errors to the number of inhalers used by an individual subject. The effect of training was evaluated after 3 months and again after 6 months. RESULTS: 50 subjects with asthma and 50 subjects with COPD were enrolled. Only 20% of subjects made no critical errors before the intervention. Subjects who were trained in the proper inhalation technique made fewer errors after 3 months (32 of 50 vs 20 of 50). The relative risk was 1.63 (95% CI 1.1-2.4, P = .01) and the number needed to treat was 3.9 (95% CI 2.2-15). Despite the improvement in the inhalation technique, we found no reduction in the number of asthma/COPD exacerbations, symptom severity, or the quality of life. After 3 consecutive months, the efficacy of the intervention decreased, and only 66% of the former responders maintained the lower ratio of errors per inhaler. After 6 months, there was no difference in the number of subjects with better inhalation technique between intervention (24 of 50) and control group (27 of 50) (P = .62). CONCLUSIONS: Although a single inhalation training leads to a reduction in the number of errors made during inhalation, it does not influence the course of asthma and COPD. The positive effect of a single inhalation technique training is temporary. (ClinicalTrials.gov registration NCT02131454.).

Mishandling of pMDI and DPI inhalers in asthma and COPD - Repetitive and non-repetitive errors.

BACKGROUND: Pharmacological treatment of asthma and chronic obstructive pulmonary disease (COPD) is based mainly on inhaled medications. There is a continuous need to examine and train patients in their inhalation technique. The objective of the presented study is to determine whether the errors which patients made during inhalations are repetitive, and therefore easier to eradicate, or rather accidental, hence require more attention and effort from the health care professionals. METHODS: It was a prospective, cohort study which included adults with asthma or COPD, who have used at least one inhaler daily on a regular basis. Inhalation technique was evaluated twice in a six months interval basing on a list of the most common errors in the inhalation technique. There was no training of inhalation skills between visits. RESULTS: There were 92 patients (46 asthmatics, 46 with COPD; median age 66 years, median duration of the disease 10 years) included into the analysis. 92% of patients made at least one error during their inhalation. Among pMDI users the most common device mishandlings were: no or too short breath-holding after inhalation (60% of the patients during the first visit; 50% during the 2nd), too rapid and too forceful inhalation (52%; 61%) and lack of exhalation before the use of the medicine (48%; 43%). Among the DPI users, the most numerous errors were: no or too short (less than 3 s) breath-holding after inhalation (62%; 55%) and slow and not forceful enough inhalation (38%; 36%). When comparing the mishandlings in the inhalation technique conducted during the first and second visit the majority of the errors conducted by the patients were repetitive. However, some errors such as too early termination of inhalation (p = 0.016), inhalation through the nose during actuation (p = 0.002) among pMDI users and lack of expiration before inhalation (p = 0.027) among DPI users, were non-permanent. CONCLUSIONS: Improper inhalation technique is very common and the majority of errors made in inhalation technique are repetitive. This emphasizes the role of an ongoing verification and training of a proper inhalation technique in all patients that are regularly treated with inhalers.