The Long QT Syndrome with Impaired Atrioventricular Conduction: A Malignant Variant in Infants

Abstract

Our findings, and similar observations by others, suggest involvement of the sinus node and the distal conduction system in this form of the LQTS. Several histologic studies have documented abnormalities within the conduction system, including apoptosis. Because of the rare occurrence and poor prognosis of the LQTS with impaired AV conduction, international guidelines for diagnosis and treatment are needed. Development of an internal cardiac defibrillator for this young age group is necessary.