Shared decision-making has become a new focus of health policy. Though its core elements are largely agreed upon, there is little consensus regarding which outcomes to prioritize for policy-mandated shared decision-making.

We report a patient with long QT syndrome who received an inappropriate implantable cardioverter-defibrillator shock due to electrical interference from a refrigerator. This electrical interference was mistakenly detected as an episode of ventricular fibrillation and ended with an inappropriate delivery of shock without any warning symptoms before.

A case of hypertrophic cardiomyopathy in the transition from childhood to adulthood, which was low risk by the conventional risk assessment model, medium risk by the adult risk prediction model, and high risk by the paediatric risk prediction model, was inserted an implantable cardioverter-defibrillator. Three years post-implantation, the patient was resuscitated with an appropriate discharge of cardioverter-defibrillator.

Despite the life-saving capabilities of implantable cardioverter-defibrillators, they may have implications on behavioural and emotional well-being and have been shown to negatively affect patients’ psychosocial functioning. Children and CHD patients with these devices are at higher risk for complications, and therefore may have higher risk of psychosocial dysfunction including depression, anxiety, and a decrease in overall quality of life. In addition, these patients may be restricted from activities, which may also contribute to psychosocial dysfunction. Recommendations published in 2015 support a more liberal approach to athletic participation in this patient population compared with previous guidelines. Approaches to limit psychosocial dysfunction include education, minimisation of shocks, and psychosocial therapy. Psychosocial dysfunction should be assessed at each clinic visit, and information regarding intervention should be provided to patients and their families as needed. Psychosocial dysfunction may be debilitating, and healthcare providers should facilitate and support normal psychosocial function by offering resources as needed.

The decision of whether to allow a young patient with an implantable cardioverter-defibrillator to continue to participate in sports is complex and multi-factorial. The positive physical and psychosocial impact of sports participation must be weighed against the potential adverse events associated with implantable cardioverter-defibrillators. Arrhythmias appear to be more prevalent in athletes and occur more frequently during physical activity or competition/practice, but there is growing evidence that device therapy is effective in athletes across a wide range of competitive sports. Failure of a device to convert a life-threatening arrhythmia, major injury from a shock, and increased lead failure have thus far not been reported in the prospective Implantable Cardioverter-Defibrillator Sports Registry, but follow-up remains relatively short. Thoughtful consideration of disease state, arrhythmia risk, and the potential dangers of device therapy during the desired sports is imperative before allowing participation. Frank discussion with children and families regarding the possibility of shocks during sports, as well as at other times, is imperative. Ongoing and future studies will help guide these decisions.

Implantable cardioverter-defibrillators effectively reduce the rate of sudden cardiac death in children. Significant efforts have been made to better characterise the indications for their placement, and over the past two decades there has been a shift in their use from secondary to primary prevention. Primary prevention includes placement in patients thought to be at high risk of sudden cardiac death before the patient experiences any event. Secondary prevention includes placement after a high-risk event including sustained ventricular tachycardia or resuscitated cardiac arrest. Although liberal device implantation may be appealing even in patients having marginal indications, studies have shown high rates of adverse effects including inappropriate device discharges and the need for re-intervention because of hardware malfunction. The indications for placement of an implantable cardioverter-defibrillator, whether for primary or secondary prevention of sudden cardiac death, vary based on cardiac pathology. This review will assist the provider in understanding the risks and benefits of device implantation in order to enhance the shared decision-making capacity of patients, families, and providers.

Local anaesthesia combined with conscious sedation is becoming a popular technique for implantation of cardioverter-defibrillator devices. Propofol was given to provide loss of consciousness during defibrillation shock administration, for induced ventricular fibrillation testing. Propofol was found to decrease QT interval and QT dispersion in two patients with idiopathic prolonged QT interval and QT dispersion. The findings of the procedure are reported.