Sudden death in an adult with a small ventricular septal defect and an aneurysmal membranous septum

Abstract

An apparently healthy man of 26 years of age suddenly died. He was known to have had a small ventricular septal defect and complete right bundle branch block from early childhood. At post-mortem examination the small ventricular septal defect was found associated with an aneurysm of the membranous septum. Histological examination showed a normal atrioventricular node and bundle, adjacent to the aneurysm. There was fibrous interruption at the commencement of the right bundle branch, which was considered the basis of the bundle branch block. It was also considered that the right bundle branch block was of the hereditary type and that this, rather than the aneurysm of the membranous septum, was responsible for the unexpected death.

Interventricular Septal Aneurysm: an Exceptional cause of Sudden Death in Young Adult

“…Cardiac rhythm and conduction disorders in patient with VSA were described in several papers [10,11]. Complete atrioventricular block, left or most frequently right bundle block were reported [4,5,9]. These complications could be explained by the compression of the conduction pathways by fibrotic tissue around the aneurysm [12].…”

“…Therapeutic management is often problematic when VSA is incidentally diagnosed in asymptomatic patient, and when discovered on autopsy the dilemma is how can it explain an "unexplained" death. Natural course of this disease remains unpredictable; even if in some cases favorable outcomes are reported without any therapeutic intervention [6,7] serious and sometimes fatal complications may occurs [8,9]. Cardiac rhythm and conduction disorders in patient with VSA were described in several papers [10,11].…”

Sudden cardiac death in younger adults: autopsy diagnosis as a tool for preventive medicine

“…Three subjects had been operated on earlier for tetralogy of Fallot (n = 2) or aortic coarctation (n = 1) and were in stable healthy condition; 3 displayed an undiagnosed obstruction of the ventricular outflow (isolated aortic stenosis; combined subvalvar, valvar, and supravalvar aortic stenosis; and supravalvar pulmonary stenosis); and 1 had an unsuspected atrial septal defect with massive volume overload in the right ventricle. Conduction system disease was diagnosed in 4 cases: Wolff-Parkinson-White syndrome with histological identification of the anomalous atrioventricular connection (n = 2), fibrosis of the His bundle associated with a small ventricular septal defect (n = 1) [19,20], and diffuse calcifications limited to the specialized conduction tissue (n = 1).…”

A ventricular septal defect restricted by the tricuspid septal leaflet and discrete subaortic membrane presenting with high-grade atrioventricular block and syncope

“…Cases with membranous ventricular septal defect and atrioventricular block become symptomatic in the 3rd or 4th decade of life. 1 , 2 , 3 In this case report, we described a 32-year-old woman with ventricular septal defect and discrete subaortic membrane without left ventricular outflow tract obstruction associated with high-grade atrioventricular block and syncope.…”