Solving the disputes concerning the congenitally malformed heart

Abstract 

It is a privilege and pleasure for me to contribute to this issue, which honours the huge contribution made to pediatric cardiology by the late Robert Freedom. In my review, I discuss the influence of Bob in temporising the numerous morphologic polemics that have continued over the past 30 years. Initially trained with Van Praagh, and steeped in the Van Praaghian codification, Bob was also able to see the significance of sequential analysis, with emphasis of the junctions between cardiac components, as emphasised by the European school of which I was part. In this review, I discuss the background to the development of the sequential segmental approach, and then show how the criticisms levelled against many of the concepts of the European school by Van Praagh himself, when responded to in appropriate scientific fashion, served to strengthen the overall analytic approach. I will focus in particular on the so-called “univentricular” heart, showing how continuing problems can be resolved by recognition of the functionally univentricular nature of lesions with one big and one small ventricle when the small ventricle is incapable of supporting one of the other circulations. I will also discuss the analysis of the organ systems in patients with visceral heterotaxy, showing how the “Morphological Method” dictates that the appendages should be the final arbiter of atrial morphology. When attention is paid to these components, it becomes evident that isomerism of the appendages is the pathognomonic feature permitting categorisation of the hearts from patients with “splenic syndromes”.

Keywords: Segmental analysis, Sequential segmental approach, Functionally univentricular heart, Isomerism, Visceral heterotaxy, Splenic syndromes