| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Cardiac Unit, Institute of Child Health, 30 Guilford St, London, WC1N 1EH United Kingdom
(Email: r.anderson{at}ich.ucl.ac.uk).
Uemura and associates [1] describe the anatomic patterns encountered in a cohort of almost 100 patients with double inlet left ventricle, with just over half of their cohort studied subsequent to death of the patient and autopsy, but with the remainder assessed in the clinical setting. As they indicate, it is usual for patients with double inlet left ventricle to be stratified according to the location of the incomplete and rudimentary right ventricle relative to the dominant left ventricle, with two-thirds of their cohort having the most common pattern in which the incomplete right ventricle is left-sided. This situation is usually taken to be the consequence of left-handed ventricular looping during development, although perhaps sensibly, the authors do not venture into the potential minefield of embryological speculation.
Only 2 of their patients with leftward right ventricles had concordant ventriculoarterial connections, a combination which the authors stress as being very rare. Most of their patients, therefore, could be placed into the three anatomic stereotypes of leftward right ventricle with discordant ventriculoarterial connections, the commonest pattern, or rightward right ventricle with either discordant or concordant ventriculoarterial connections. These latter two stereotypes were present in almost equal numbers in their series.
The pattern with rightward right ventricle and concordant ventriculoarterial connections has historical connotation, being first described in 1842 by Andrew Holmes [2], and now universally recognized as the Holmes heart. As much as one-quarter of the cohort, nonetheless, could not be fitted into these stereotypical patterns. This was because of unexpected ventriculoarterial connections, such as double outlet from the dominant left or the incomplete right ventricle, because of the presence of pulmonary atresia, or else because the relationship of the arterial trunks was not anticipated for the given ventriculoarterial connection. Their study confirms that patients with so-called complex lesions always require full sequential segmental analysis, with attention always paid separately to the connections present between the cavities of the adjacent cardiac segments and the interrelationships of their component parts.
All of their patients bar one had usual atrial arrangement, and presumably a double inlet through 2 separate atrioventricular valves. This suggests a degree of selection in their series, because in the analysis made by Franklin and colleagues [3] of patients presenting clinically in the first year of life, only 113 of 136 patients with double inlet left ventricle had a usual atrial arrangement, with 19 having isomeric atrial appendages, or so-called visceral heterotaxy. Of this latter series, 42 of 136 had a common atrioventricular valve, another feature of major clinical significance.
It is the 2 patients described by Uemura and colleagues [1] with leftward right ventricles and concordant ventriculoarterial connections that arouse my own particular interest. They suggest that these patients could be described as having "anatomically corrected malposition." In fact, when there are spiralling arterial trunks, with the pulmonary trunk arising to the left of the aorta, if a descriptive term is to be used, the arrangement would be called "isolated ventricular inversion." This is because when there is usual atrial arrangement and usual spiralling of concordantly connected arterial trunks, it is the leftward location of the right ventricle that is isolated in terms of intersegmental relationships.
When the arterial trunks are concordantly connected in the setting of double inlet left ventricle and leftward incomplete right ventricle, albeit a very rare event, it is more usual for the trunks to exit from the heart in parallel fashion, with the aorta to the left. It is this latter combination that could then be described as anatomically corrected malposition. We recently described several autopsied specimens with the latter arrangement [4]. The cases described by Uemura and colleagues [1], with spiralling arterial trunks, leftward incomplete right ventricle, and concordant ventriculoarterial connections constitute an exceedingly rare combination that, to the best of my knowledge, has not previously been described.
 |
References |
|---|
 Top References |
|---|
Related Article
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| ANN THORAC SURG | ASIAN CARDIOVASC THORAC ANN | EUR J CARDIOTHORAC SURG |
| J THORAC CARDIOVASC SURG | ICVTS | ALL CTSNet JOURNALS |
