Prescribing an automated external defibrillator for children at increased risk of sudden arrhythmic death.

BACKGROUND: Automated external defibrillators can be life-saving in out-of-hospital cardiac arrest. OBJECTIVE: Our aim was to review our experience of prescribing automated external defibrillators for children at increased risk of sudden arrhythmic death. METHODS: We reviewed all automated external defibrillators issued by the Scottish Paediatric Cardiac Electrophysiology Service from 2005 to 2015. All parents were given resuscitation training according to the Paediatric Resuscitation Guidelines, including the use of the automated external defibrillator. RESULTS: A total of 36 automated external defibrillators were issued to 36 families for 44 children (27 male). The mean age at issue was 8.8 years. Diagnoses at issue included long QT syndrome (50%), broad complex tachycardia (14%), hypertrophic cardiomyopathy (11%), and catecholaminergic polymorphic ventricular tachycardia (9%). During the study period, the automated external defibrillator was used in four (9%) children, and in all four the automated external defibrillator correctly discriminated between a shockable rhythm - polymorphic ventricular tachycardia/ventricular fibrillation in three patients with one or more shocks delivered - and non-shockable rhythm - sinus rhythm in one patient. Of the three children, two of them who received one or more shocks for ventricular fibrillation/polymorphic ventricular tachycardia survived, but one died as a result of recurrent torsades de pointes. There were no other deaths. CONCLUSION: Parents can be taught to recognise cardiac arrest, apply resuscitation skills, and use an automated external defibrillator. Prescribing an automated external defibrillator should be considered for children at increased risk of sudden arrhythmic death, especially where the risk/benefit ratio of an implantable defibrillator is unclear or delay to defibrillator implantation is deemed necessary.

Implantation of a fully subcutaneous ICD in children.

The subcutaneous implantable cardioverter defibrillator (S-ICD) from Cameron Health (San Clemente, CA, USA) does not require a lead to be placed on or in the heart. Such a device, being subcutaneous, has potential benefits in children who require ICDs where problems largely relate to transvenous or epicardial leads and inappropriate shocks. The S-ICD was approved for use in Europe in June 2009 and recently a study commenced to acquire data in 330 patients in order to submit to the FDA. We shall describe the implantation of the S-ICD in two children aged 10 and 12 years at our institution.

Friedreich's ataxia-associated childhood hypertrophic cardiomyopathy: a national cohort study.

OBJECTIVE: Hypertrophic cardiomyopathy (HCM) is an important predictor of long-term outcomes in Friedreich's ataxia (FA), but the clinical spectrum and survival in childhood is poorly described. This study aimed to describe the clinical characteristics of children with FA-HCM. DESIGN AND SETTING: Retrospective, longitudinal cohort study of children with FA-HCM from the UK. PATIENTS: 78 children (<18 years) with FA-HCM diagnosed over four decades. INTERVENTION: Anonymised retrospective demographic and clinical data were collected from baseline evaluation and follow-up. MAIN OUTCOME MEASURES: The primary study end-point was all-cause mortality (sudden cardiac death, atrial arrhythmia-related death, heart failure-related death, non-cardiac death) or cardiac transplantation. RESULTS: The mean age at diagnosis of FA-HCM was 10.9 (±3.1) years. Diagnosis was within 1 year of cardiac referral in 34 (65.0%) patients, but preceded the diagnosis of FA in 4 (5.3%). At baseline, 65 (90.3%) had concentric left ventricular hypertrophy and 6 (12.5%) had systolic impairment. Over a median follow-up of 5.1 years (IQR 2.4-7.3), 8 (10.5%) had documented supraventricular arrhythmias and 8 (10.5%) died (atrial arrhythmia-related n=2; heart failure-related n=1; non-cardiac n=2; or unknown cause n=3), but there were no sudden cardiac deaths. Freedom from death or transplantation at 10 years was 80.8% (95% CI 62.5 to 90.8). CONCLUSIONS: This is the largest cohort of childhood FA-HCM reported to date and describes a high prevalence of atrial arrhythmias and impaired systolic function in childhood, suggesting early progression to end-stage disease. Overall mortality is similar to that reported in non-syndromic childhood HCM, but no patients died suddenly.

Lead-induced tricuspid stenosis--successful management by balloon angioplasty.

We describe a case of tricuspid stenosis in a young woman 11 years after endocardial ventricular lead implantation. The cause of the stenosis appears to have been perforation of the septal leaflet by the lead at the time of insertion. We further describe successful management with percutaneous balloon valvuloplasty without the need for lead removal.

Prostaglandin E2 after septostomy for simple transposition.

In simple transposition of the great arteries (sTGA), balloon atrial septostomy is performed prior to arterial switch to improve mixing of systemic and pulmonary circulations. Following septostomy, some patients are also given prostaglandin E2 (PGE2) until surgical repair. The aims of our study were to identify how often PGE2 is given after septostomy, the indications for starting PGE2, and the effect this has on postoperative outcome. The study was a retrospective review of infants born with sTGA between 2000 and 2005, who underwent arterial switch at Yorkhill Children's Hospital, Glasgow. Over a 5-year period, 26 infants (16 male) with sTGA underwent septostomy. There was a significant rise in mean oxygen saturation following septostomy (mean, 61.4 +/- 11.5% before, 81.5 +/- 9.4% after; p < 0.05). Four of 26 (15%) did not receive PGE2 at all (group 1) and 8 of 26 (30%) received PGE2 before but not after septostomy (group 2). A total of 14 of 26 infants (54%) were given PGE2 following septostomy. This comprised 11 who received PGE2 before and after septostomy (group 3) and 3 who did not receive PGE2 prior to septostomy but did after (group 4). Groups 2 and 3 were compared directly, as they both received PGE2 before septostomy. In group 3, oxygen saturations were lower when PGE2 was started compared with saturations immediately after septostomy (45 +/- 23.6% vs. 80 +/- 10.3%; p < 0.05). Groups 2 and 3 showed no difference in atrial gap after septostomy (9.4 +/- 3 vs. 8 +/- 1 mm; p > 0.05). Fifty percent of infants in group 3 underwent echocardiography prior to restarting PGE2, which revealed a patent arterial duct in all but one patient. Despite PGE2, Group 3 had lower saturations at arterial switch compared with Group 2 (71 +/- 14% vs. 82 +/- 8%; p < 0.05). No difference was observed between group 2 and group 3 with regard to length of cardiopulmonary bypass (group 2, 173 +/- 101.4 min, vs. group 3, 157.9 +/- 42.1 min; p > 0.05). However, the Intensive Care Unit stay was longer for patients who received PGE2 following septostomy (8.5 +/- 10.3 vs. 5 +/- 0.93 days; p < 0.05). Total postoperative stay was also longer for infants who received PGE2 after septostomy (26.8 +/- 14.3 vs. 16.8 +/- 6.3 days; p < 0.05). In conclusion, the use of pulse oximetry has led to an increase in the administration of PGE2 after septostomy. PGE2 administration was associated with a longer ICU stay. The association between administration of PGE2 and longer postoperative stay supports the approach of early surgical repair with minimal preoperative medical intervention.

Comparison of a smartphone-based ECG recording system with a standard cardiac event monitor in the investigation of palpitations in children.

BACKGROUND: The AliveCor (Kardia) monitor attaches to a smartphone and allows a single-lead ECG to be recorded during symptoms. In 2016, we introduced the use of this smartphone device for investigating palpitations, without syncope, in children. The aim of our study was to review our experience with the smartphone device, comparing it with our previous standard conventional approach to cardiac event monitoring using the Cardiocall monitor, which uses skin electrodes and is given for a finite period. METHODS: Over a period of 24 months, 80 smartphone monitors were issued and compared with the most recent 100 conventional event monitors. The number of ECG recordings received, arrhythmias documented, quality of ECG recordings and patient satisfaction were evaluated. RESULTS: Median patient age was 11 years in the smartphone monitor group compared with 10 years in the conventional group. Seventy-nine of 80 (98%) patients with a smartphone monitor sent an ECG recorded during symptoms, compared with 62/100 (62%) from the conventional group. A total of 836 ECG recordings were sent from the smartphone monitors compared with 752 from the conventional group. Eight per cent of ECG recordings in each group were of inadequate quality for analysis. Twenty of 80 (25%) patients with a smartphone monitor had documented tachyarrhythmia compared with 6/100 (6%) patients with the conventional monitor (p<0.001). On comparison with the conventional approach, the smartphone monitor outperformed with respect to diagnostic yield and patient satisfaction. CONCLUSIONS: A smartphone-based event monitor allows simple, effective, long-term ECG event monitoring in children that is highly acceptable to the patient and parent.