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and medical care team By incorporating extensive education in regard to feeding and growth, infection risks, and long-term complications when inpatient, clinicians should prepare families for successful management of their child at home and in the ambulatory setting Annotated References Ivemark BI Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood; an analysis of the heart malformations in the splenic agenesis syndrome, with fourteen new cases Acta Paediatr Suppl 1955;44:7–110 Initial manuscript describing what became known as asplenia and now contemporarily is understood to be right isomerism Van Praagh R, David I, Wright GB, Van Praagh S Large RV plus small LV is not single RV Circulation 1980;61:1057–1059 Manuscript that stresses the importance of how the description of cardiac morphology needs to be described in relation to features that are constant This is a notion of particular importance in isomerism and demonstrates why the atrial appendages should be used as the basis of segregating isomerism Uemura H, Ho SY, Devine WA, Anderson RH Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both Am J Cardiol 1995;76:846–849 A necroscopy study characterizing the features of hearts with isomerism, with particular focus on whether splenic status or atrial appendage morphology is more useful in segregating isomerism The first study to demonstrate that more defined, homogenous segregation can be achieved based on atrial appendage morphology versus splenic morphology Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy Ann Thorac Surg 1995;60:561–569 A necroscopy study investigating the venoatrial connections in hearts with isomerism This study defines the superior and inferior caval vein connections in these hearts as well as the pulmonary venous connections, highlighting the differences in venoatrial connections between right and left isomerism Jacobs JP, Anderson RH, Weinberg PM, et al The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy Cardiol Young 2007;17(suppl 2):1–28 This is an effort by a large working group attempting to describe a uniform system by which to describe so-called heterotaxy, now understood to be better described as isomerism Van Praagh R, Van Praagh S Atrial isomerism in
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