Atresia of the right atrioventricular orifice with atrioventricular concordance.

Dickinson, D F; Wilkinson, James L.; Smith, Audrey; Anderson, Robert H.

doi:10.1136/hrt.42.1.9

Cited by 19 publications

(8 citation statements)

References 14 publications

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“…When we consider the distribution of conducting tissue within the ventricular mass, our findings in hearts with absent right atrioventricular connection are virtually identical with those previously reported in univentricular hearts of left ventricular type with double inlet connection (Anderson et al, 1974;Bharati and Lev, 1975). They differ considerably from hearts with atrioventricular concordance but an imperforate tricuspid membrane (Dickinson et al, 1979). In these latter specimens the conducting tissue is directly comparable with that seen in isolated ventricular septal defect (compare Fig.…”

Section: Discussionsupporting

confidence: 87%

“…They recognised, however, that in a small num-1RHA is supported by the Joseph Levy Foundation together with the British Heart Foundation. 1 ber of cases an imperforate tricuspid membrane interposed between the right atrium and a formed right ventricle Dickinson et al, 1979). On the other hand, Bharati et al (1976) considered the anterior chamber in 'tricuspid atresia' to be a true right ventricle in all cases.…”

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Atrioventricular conduction tissues in univentricular hearts of left ventricular type with absent right atrioventricular connection ('tricuspid atresia').

Dickinson¹,

Wilkinson²,

Smith³

et al. 1979

Heart

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SUMMARYThe atrioventricular conduction tissues in 14 hearts with absence of the right atrioventricular connection ('tricuspid atresia') were studied by serial sectioning. In all cases, the atrioventricular node lay in the floor of the right atrium, posterior to the tendon of Todaro, but in all but one case it extended anteriorly either medial or lateral to the insertion of the tendon in a way not seen in the normal heart. The common bundle frequently arose from the anterior or lateral extensions of the node and, after penetration of the central fibrous body, came to lie on the right wall of the main ventricular chamber at the point of junction with the septum separating this chamber from the outlet chamber. In all but 3 cases, branching of the bundle occurred posterior to the outlet foramen. The distribution of the left bundle characteristically favoured the posterior part of the main chamber and the right bundle passed inferior to the outlet foramen to ramify in the trabecular zone of the outlet chamber.Although these findings differ in some respects from those previously described in univentricular hearts with an outlet chamber and 2 atrioventricular valves, we believe that they are consistent with our view that the majority of hearts with absence of the right atrioventricular connection come within the category of the univentricular heart.In recent years, the outlook for patients with atresia or absence of the right atrioventricular orifice has been improved by the use of corrective surgical procedures such as that developed by Fontan and Baudet (1971). During such procedures, the conducting tissue may be at risk during closure of the atrial septal defect, during closure of the outlet foramen, and when enlargement of the outlet foramen is required because the aorta arises from the outlet chamber. The disposition of conducting tissue also has implications for the morphology and classification of the anomaly.Anderson and his colleagues (1977) have expressed the view that atresia (or absence) of the right atrioventricular orifice is, in most cases, associated with the ventricular morphology of a univentricular heart, usually with an anterior rudimentary chamber. They recognised, however, that in a small num-1RHA is supported by the Joseph Levy Foundation together with the British Heart Foundation.

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confidence: 87%

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confidence: 99%

Atrioventricular conduction tissues in univentricular hearts of left ventricular type with absent right atrioventricular connection ('tricuspid atresia').

Dickinson¹,

Wilkinson²,

Smith³

et al. 1979

Heart

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“…The right ventricle in the former lesion may be quite large, possessing a normally developed inlet component [5] . This becomes important in the presence of ventriculoarterial concordance since the right ventricle can be used to Pediatric Cardiology Vol .…”

Section: Discussionmentioning

confidence: 99%

“…In this condition the blind-ending right atrium is in potential communication with a normally constituted, albeit hypoplastic, right ventricle via an imperforate tricuspid valve which may either exhibit the features of an Ebstein's anomaly [9, 111, or consist of a fibrous membrane [ 1,5] . A rudimentary valvar tension apparatus may be present, as occurred in our two cases, being attached to the ventricular side of the imperforate tricuspid valve .…”

Section: Discussionmentioning

confidence: 99%

“…Yet there are very rare cases in which the right atrium is in potential communication with the right ventricular chamber, the two chambers being separated by an imperforate tricuspid valve [1, 9,11] . Hearts with this anomaly, as opposed to classic tricuspid atresia where the right ventricular chamber lacks an inlet portion, exhibit a normally formed right ventricle which possesses recognizable inlet, trabecular, and outlet components and which may in some instances have a large ventricular cavity [5] .Preoperative recognition of this lesion is important from a surgical point of view in the presence of Address reprint requests to : Giancarlo Crupi, MD, Department of Cardiac Surgery, Ospedali Riuniti, 1-24100 Bergamo, Italy .Pediatric Cardiology © Springer-Verlag 1984 ventriculoarterial concordance . Indeed, the possibility of using the right ventricle to support the pulmonary circulation should be carefully evaluated in planning surgical treatment for this lesion .…”

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Tricuspid atresia with imperforate valve: Angiographic findings and surgical implications in two cases with AV concordance and normally related great arteries

et al. 1984

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The angiographic and intraoperative findings are described in two children with imperforate tricuspid valve with atrioventricular and ventriculoarterial concordance who underwent a successful Fontan operation. It is important from a surgical point of view to distinguish this rare variant of tricuspid atresia from the more classic form with absent atrioventricular connection. The characteristic feature of hearts with imperforate tricuspid valve is the presence of a normally formed right ventricle which, in the presence of ventriculoarterial concordance, can be used to support the pulmonary circulation. When the imperforate valve is large, it may be possible to remove it, and restore atrioventricular continuity. Alternatively, an atrioventricular type of Fontan repair using a valved conduit should be considered as the treatment of choice in the presence of a suitable pulmonary arterial tree and pulmonary valve.

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2003

The Natural and Modifed History of Congenital Heart Disease

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Atresia of the right atrioventricular orifice with atrioventricular concordance.

Cited by 19 publications

References 14 publications

Atrioventricular conduction tissues in univentricular hearts of left ventricular type with absent right atrioventricular connection ('tricuspid atresia').

Atrioventricular conduction tissues in univentricular hearts of left ventricular type with absent right atrioventricular connection ('tricuspid atresia').

Tricuspid atresia with imperforate valve: Angiographic findings and surgical implications in two cases with AV concordance and normally related great arteries

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