VENTRICULAR ARRHYTHMOGENESIS AND SUDDEN DEATH: Histologic Correlates in Two Young Females

Abstract

Two female Pts. ages 5 and 14, with history of ventricular dysrrhythmia (VD) died suddenly. Pt I, had post-natal chaotic AV block and a prolonged QT interval. The infant required transvenous ventricular pacing which controlled the arrhythmia by overdrive suppression. A permanent epicardial unipolar VVI pacemaker was implanted at one week of age. The Pt had a normal hemodynamic study and complete RBB block pattern with left axis deviation with pacing. Febrile seizures occurred at age 5. Holter studies demonstrated normal pacemaker function and capture and no ventricular ectopy. Two weeks prior to her sudden death, a short self terminated run of VT was noted. Autopsy revealed an enlarged heart. Conduction system (CS) revealed chronic, patchy infiltration of cells in the myocardium and neuritis, with fibrosis of the bundle and the beginning of the bundle branches. Pt 2, had a 6 year history of paroxysmal VT, normal EP studies, was asymptomatic and was free of VD on proprananol. A brother with an identical condition had documented clinical and EP evidence of catechol induced ventricular premature beats and VT. Autopsy revealed cardiomegaly, a left sided His bundle, and markedly dilated veins pressed on the AV node. A conal muscle from the right side pressed on the bifurcating bundle, and the RBB was intramyocardial. In summary, arrhythmogenicity could have been triggered by changes in the CS resulting in sudden death in both pts.

Log in or create a free account to read this content

Gain free access to this article, as well as selected content from this journal and more on nature.com

Access through your institution

or

Sign in or create an account

Continue with Google

Continue with ORCiD