Univentricular Heart

TLDR

Since 1699, the univentricular heart has fascinated clinicians, provoking debates on embryology, nomenclature, physiology, and hemodynamics, and inspiring innovative surgical and interventional strategies. This report reviews the nomenclature, classification, epidemiology, genetics, physiology, clinical features, diagnostics, therapy, and postoperative sequelae of the univentricular heart. The review targets adult cardiologists, while also addressing issues relevant to neonates and children.

Abstract

As early as 1699, Chemineau described a heart composed of 2 auricles but only 1 ventricle. 1 The univentricular heart has since fascinated the medical community. Unique in its complexity and scope, the univentricular heart has sparked intense debates about embryology and nomenclature, challenged our understanding of cardiovascular physiology and hemodynamics, and inspired some of the most creative surgical and interventional approaches in human history. The present report provides an overview of the nomenclature and classification of the univentricular heart, epidemiology and pathological subtypes, genetic factors, physiology, clinical features, diagnostic assessment, therapy, and postoperative sequelae. Although the present report touches on issues applicable to neonates and children with univentricular hearts, the focus is on information of interest and relevance to the adult cardiologist.