Controversies in the description of congenitally malformed hearts anderson robert h becker anton e. Is transposition a defect of laterality? Anderson RH, Stickley J 2019-02-03

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Sequential segmental analysis of congenitally malformed hearts: Advances for the 1990s

controversies in the description of congenitally malformed hearts anderson robert h becker anton e

This produces an atrioventricular connection that is biventricular but mixed. All three criteria need to be fulfilled to satisfy these definitions. These sagittal sections from a mouse on embryonic day 11. This is not because of a lack of awareness of the potential significance of some areas of atrial myocardium being different from the larger part of the chamber myocardium. Anatomic types of single orcommon ventricle in man: morphologic and geometric aspects of sixtynecropsied cases. We had not fully understood,however, the subtleties of the segmental approach.

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CONGENITALLY

controversies in the description of congenitally malformed hearts anderson robert h becker anton e

Pulmonary atresiawith intact ventricular septum: management of, and outcomes for, a cohortof 210 consecutive cases. Big Bobwas a giant inall respects. Bethat as it may, in all patients with visceral heterotaxy it is nowclear that the evidence supporting the existence of isomerism ofthe appendages is unequivocal. On the basis of the staining properties for connexin40 and atrial natriuretic factor, therefore, it is possible to distinguish between nodal and working myocardium. Thus, in thesystem as initially described, any heart arranged with {S, D, }can have atrioventricular concordance, irrespective of whether theatrial chambers are joined to their appropriate ventricles, to thesame ventricle, or whether there is atrioventricular valvar atresia. In such hearts, there is peristaltic propulsion of the blood, with the inlet of the heart functioning as the dominant pacemaker, although its cells are morphologically indistinguishable from the remaining myocardial cells. In the remainder of this review, we will restate the criteria that, in our opinion, serve as the gold standard for anatomical definition of the conducting structures in the postnatal heart.

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controversies in the description of congenitally malformed hearts anderson robert h becker anton e

Anderson has now been researching the conduction tissues of the heart for more than 40 years. It is an undeniable fact that description of the development of specialized internodal tracts acts as a red flag to the first author of this review. Even within the myocardium that we label as being working, there is significant heterogeneity in terms of morphology, ultrastructure, and gene expression. As was emphasized in the contribution to the Novartis symposium Moorman and Christoffels, , the heart in primitive chordates is no more than a myocardial mantle enveloping the ventral aorta. The cushion isbecoming muscularised, and will eventually form the subpulmonaryinfundibulum. The extent of the primary nodal myocardium in these early stages, however, is significantly larger than the areas of all the tissue recognizable as nodal in the postnatal heart within the anatomical criteria. Two cases of congenitalasplenia with isomerism of the cardiac atria and the sinoatrial nodes.

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Solving the disputes concerning the congenitally malformed heart

controversies in the description of congenitally malformed hearts anderson robert h becker anton e

It seemed to us that our proposals were no more than a directextension of the original system. Christoffels is a member of the staff of the Department of Anatomy and Embryology in the Academic Medical Center at the University of Amsterdam and an established investigator in the Molecular Cardiology Program on Heart Failure funded by the Netherlands Heart Foundation. Having defined the ventricularmass in this fashion, it is an inescapable fact that the atrialchambers are connected to their respective ventricles across the Fig. Those who cite problems of lateralisation as underscoring the finding of discordant ventriculo-arterial connections are seeking to establish the genetic cues that might underscore the pathogenetic mechanisms. 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 withsplenic syndromes. Insulated pathways running through the atrial myocardium between the cardiac nodes simply do not exist, neither prenatally nor postnatally.

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Is transposition a defect of laterality? Anderson RH, Stickley J

controversies in the description of congenitally malformed hearts anderson robert h becker anton e

At thattime, however, it was usual to stratify cardiac arrangement onthe basis of asplenia and polysplenia. The working myocardial cells of the chambers, in contrast, are richly endowed with connexin40 at their junctions and also contain demonstrable quantities of atrial natriuretic factor Figure. It is alsouniversally recognised that visceral heterotaxy itself segregatesinto two discrete sub-sets. This can only be achieved by careful examination of serial sections. The criteria suggested by Monckeberg and Aschoff , therefore, were designed for the distinction of insulated tracts that conducted more rapidly than the adjacent myocardium. Thus, the questions that need to be resolved by the embryologists and molecular biologists are the mechanisms of transformation of the primary myocardium into the definitive conduction tissues as defined within the criteria of Aschoff and Monckeberg.

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CONGENITALLY

controversies in the description of congenitally malformed hearts anderson robert h becker anton e

This panel shows the rudimentary and incomplete right ventricle from acase of classical tricuspid atresia, such as illustrated in four-chamber plane inFig. Inthe cases with most severe mural hypertrophy, therefore, theventricle, although still possessing its three component parts, had acavity formed effectively by the hypoplastic inlet Fig. We argue that application of these principles will distinguish between those controversies which are semantic as opposed to those reflecting persisting scientific disagreements. Nomenclature and classification: Morphologic and segmentalapproach to diagnosis. In this review, we restate the criteria of Aschoff and Monckeberg, then discuss their relevance to the developmental issues, emphasizing that all myocardial cells have the potential to conduct. We now recognise that it is the atrioventricular connections thatare univentricular, not only in the double inlet ventricle, but alsoin hearts with the absence of one atrioventricular connection Fig.

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controversies in the description of congenitally malformed hearts anderson robert h becker anton e

Instead, it is a problem with ventricular looping. This iscertainly one area where we still have major differences ininterpretation, rather than simple problems in semantics. For Bob, however, it was his transfer to Toronto,accompanying Dick Rowe from Hopkins, which gave him theopportunity to develop his own concepts of cardiac morphology. As has been pointed out by two of us Moorman and Christoffels, , discussions of these tracts can still serve as a red flag to a bull, but only when the importance of the tracts is placed in a clinical rather than a developmental context. Had we initially used the description we now the right and left atriums. Controversies in analysing the congenitally malformedheart During the course of our careers, Bob and I have writtenextensively on the best way of describing the congenitally Fig. These sagittal sections of a mouse embryo from day 9.

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controversies in the description of congenitally malformed hearts anderson robert h becker anton e

It is salutary, however, to note that, at the birth of surgical repair of congenitally malformed hearts, it was widely believed that the heart lacked any discrete conduction system Glomsett and Glomsett, ,. Thus, while the embryologist or molecular biologist might claim with some justification that cardiac anatomy has proven to be a hindrance rather than a help in understanding normal cardiac development Moorman and Christoffels, , the cardiac surgeons who now repair congenitally malformed hearts on a daily basis throughout the world are well aware of the need for anatomical criteria that permit recognition of the location of these conduction tissues in the postnatal heart. The significance of ventriculo-coronary arterial communications in the setting of pulmonary atresia withan intact ventricular septum. Inthis review, I discuss the background to the development of the sequential segmental approach, and then show how the criticisms levelled against hen responded to in appropriate scientific fashion, served to strengthen theRe Solving the disputes concerninglate Robert Freedom. The primary cells at the inlet of the linear heart tube are then able to generate the sinus impulse, while the presence of a ring of atrioventricular primary myocardium is able to induce atrioventricular delay Moorman and Christoffels,. The ventricle itself,nonetheless, self-evidently retains its three component parts. Itseemed to us, therefore, that like the lungs and bronchuses, theatrial chambers might also be isomeric in patients with visceralheterotaxy.

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Sequential segmental analysis of congenitally malformed hearts: Advances for the 1990s

controversies in the description of congenitally malformed hearts anderson robert h becker anton e

In order to appreciate the full significance of their analysis, we should remember that both of these issues were the source of significant previous controversies amongst those who investigate and treat patients with congenitally malformed hearts. To cite but one example, it took littleconvincing for him to change the title of one chapter in hisangiography book from single ventricle to double inlet ven-tricle. In essence, these giants of German pathology established definitions for recognition of the discrete conducting tracts that exist within the human heart. Normal and abnormal structure of theventriculo-arterial junctions. His own contributionswere enormous, not only to paediatric cardiology but also tocardiac pathology. As my memory recalls, I first met Bobin the mid 1970s, when Anton Becker and I were visiting centresin North America specialising, on the one hand, in cardiacarrhythmias and, on the other hand, in the diagnosis and treat-ment of congenital cardiac malformations. The molecular makeup of the pulmonary venous myocardial sleeves nonetheless argues against a specialized nature of this region.

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