Isolated Non-Compacted Right Ventricular Myocardium with Severe Pulmonary Hypertension

Abstract

BACKGROUND: There have been only at least seven cases of isolated right ventricular non-compaction reported in literature, hence, there are no definite criteria for diagnosis and recommendations for management of these patients. The reported clinical manifestations include heart failure, arrhythmias and cardioembolic events. Left ventricular and biventricular non-compaction are likewise rare but more common than isolated right ventricular non-compaction. Pulmonary hypertension is associated with biventricular non-compaction more commonly but not with isolated right ventricular non-compaction.