Two Rare Cases of Septal, Annular, Valvular, and Ventricular Disorders

Abstract

Ventricular noncompaction is thought to occur due to the arrest of maturation of the myocardium during fetal development. It may be confined to one or both ventricles. The spectrum of noncompaction varies from an incidental finding to severe systolic and diastolic dysfunction or may manifest as ventricular arrhythmia and thromboembolism. It is known to be associated with congenital heart diseases (CHDs). Annular disjunction is an established morphological entity involving the mitral annulus commonly; although uncommon, it may also involve the tricuspid annulus. Annular disjunction is also commonly associated with atrioventricular (AV) valve regurgitation of various degrees. Association of annular disjunction and AV valve prolapse is known to be associated with ventricular tachyarrhythmia and even sudden cardiac death. The combination of CHD, bi-ventricular non-compaction, annular disjunction, and AV valve prolapse with regurgitation is extremely rare; each of these conditions will be additive to worsen prognosis. Here is a report of two such cases of rare combinations of widely varying morphological features. Whether these morphological alterations are coincidental or a link exists is speculative.