Sudden cardiac arrest in the young, the Wolfson experience

Introduction: Sudden cardiac arrest in an otherwise healthy, young patient is a challenge both in diagnosis and management. We describe our institution`s experience in this field.

Materials and Methods: Patients under the age of 30 presenting to our center after surviving an out of hospital resuscitation for ventricular fibrillation (VF) without history of cardiac disease were included. All underwent an extensive workup and then implantation of an implantable automatic cardio defibrillator (ICD), followed by home monitoring.

Results: In the years 2018-2020, 5 healthy young patients were admitted after resuscitation from ventricular fibrillation. Their ages ranged 15-22 years (average 17). In 4 cases, the baseline ECG provided clues to the final diagnosis. Coronary malformations were ruled out in all. An advanced workup including echocardiography, magnetic resonance imaging, pharmacological challenge and genetic sequencing provided the diagnosis of all: two with hypertrophic cardiomyopathy, one with Brugada Syndrome, one with catecholminergic polymorphic ventricular tachycardia and one with early repolarization syndrome. All were implanted with an ICD. At follow up, two suffered shocks due to poor medical compliance, one appropriate and one inappropriate.

Conclusions: In this challenging group of patients, a diagnosis can be made based on the initial ECG and selected, advanced testing. This diagnosis is critical to enable personalized treatment and cascade testing of family members.