Malignant Ventricular Arrhythmia With Apical Biventricular Noncompaction

Ventricular tachycardia (VT) arising from the right ventricle outflow tract is the most common cause of VT in children with a structurally normal heart. It often presents as a monomorphic VT that is usually amenable to ablation during an electrophysiology (EP) study. VT in children is typically idiopathic and benign but carries a risk for the degeneration of the arrhythmia into ventricular fibrillation or can result in sudden cardiac death. We present a unique case of a patient with a history of recurrent palpitations and chest tightness who was found to have a significant burden of malignant ventricular arrhythmias in the presence of apical biventricular noncompaction and preserved ventricular systolic function. The patient was referred for an EP study to assess for ablation if a discrete focus could be identified. During the EP study, the arrhythmia degenerated into ventricular fibrillation that required prompt defibrillation with a manual defibrillator and placement of a single-chamber transvenous implantable cardioverter defibrillator (ICD). This case report highlights how physicians should be mindful that although most cases of VT in children are idiopathic and benign, there is a risk of degeneration of the arrhythmia into ventricular fibrillation or resulting in sudden cardiac death. Prompt recognition of concerning VT with the involvement of EP in the patient's care is important.