Isolated noncompaction of ventricular myocardium associated with fatal ventricular fibrillation

A female infant with isolated noncompaction of ventricular myocardium who d eveloped ventricular tachyarrhythmia is described. Wolff-Parkinson-White sy ndrome was shown by electrocardiography. At 9 months of age, the patient su ddenly developed cardiac arrest. Electrocardiography following resuscitatio n with DC cardioversion demonstrated sinus rhythm without delta wave. The Q T interval was normal. Frequent premature ventricular captures caused ventr icular fibrillation. DC cardioversion was necessary to terminate frequent a ttacks of ventricular fibrillation until the introduction of beta Blockers and lidocaine. Two-dimensional echocardiogram confirmed the diagnosis of is olated noncompaction of ventricular myocardium. Three months later, the pat ient died of ventricular fibrillation during respiratory syncytial viral in fection.