Morphologic features of the uniatrial but biventricular atrioventricular connection (original) (raw)
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Straddling atrioventricular valve with absent atrioventricular connection. Report of 10 cases
Heart, 1982
Six hearts with absent right connection and four hearts with absent left connection in association with straddling of an atrioventricular valve are described. Their categorisation is made easier by adopting a segmental approach. The conduction tissue was studied in two hearts and the arrangements were unusual. Straddling of an atrioventricular valve may be defined as the arrangement in which its tension apparatus is attached to both sides of the ventricular septum.' 2 When defined as such, the most frequent example of a straddling valve is an atrioventricular septal defect with a common atrioventricular orifice ("complete endocardial cushion defect" or "complete atrioventricular canal malformation"), though some authorities question whether "straddling" should be used in this context.34 Less frequent are the hearts in which there are two atrioventricular valves and either the right valve or the left valve, or, exceptionally, both valves, have their tension apparatus attached to both sides of the septum.2-4 These hearts pose a major problem in surgical treatment.' Hearts have also been reported, however, in which either the right or left valve straddles when the other valve is atretic. These latter hearts are exceedingly rare,3 5-8 but they pose particular problems in classification, particularly concerning their relation to common atrioventricular orifice.9 We have now examined 10 such hearts from *RHA and FJM are supported by the British Heart Foundation together with the Joseph Levy and Vandervell Foundations, respectively; and SM was a Visiting Fellow from Chaim Sheba Medical Centre, Tel Aviv, Israel.
The 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.
Absence of one atrioventricular connexion — a terminologic problem
International Journal of Cardiology, 1991
We present a patient in whom the heart was right-sided, with usual atrial position, absence of the right-sided atrioventricular connexion, and a single outlet via the aorta from a dominant morphologically right ventricle. We use the case to illustrate potential problems in the terminology as used to describe the absence of one atrioventricular connexion. Our case shows why absence of one atrioventricular connexion must he classified according to the side of the missing connexion, and not the anatomy of the atrial or ventricular chambers involved. To simplify the approach to certain hearts, the use of the concept of one concordant or discordant atrioventricular connexion may be justified when the other atrioventrkular connexion is absent.
The spectrum of double-outlet right atrium including hearts with three atrioventricular valves
European Journal of Cardio-Thoracic Surgery, 2012
Double-outlet right atrium (DORA) is characterized by simultaneous right atrial emptying into both ventricles. Ventriculoatrial septal malalignment is the cardinal morphological feature. Three cases are presented to depict two major types of DORA-DORA with a malaligned atrial septum and DORA with a malaligned ventricular septum. We describe two subtypes of each form of DORA: DORA with a malaligned atrial septum presents with either a common atrioventricular (AV) junction (guarded by a common AV valve) or with a single AV junction (due to the absence of the left AV junction). DORA with a malaligned ventricular septum may be associated with a right ventricle (RV) that is adequate for biventricular repair or a severely hypoplastic RV not compatible with biventricular repair. DORA with a malaligned ventricular septum is closely related to typical straddling of the tricuspid valve. Peculiarly, DORA with a malaligned ventricular septum presents three AV valves at the AV junction and is associated with an abnormal disposition of the AV conduction axis. Clear understanding of the morphology of these lesions is important in preventing a surgical misadventure at the crux of the heart.
Morphologic Analysis of Common Atrioventricular Valves in Patients with Right Atrial Isomerism
Pediatric Cardiology, 1997
The objective of the study was to examine the relation between the morphology of the common atrioventricular valve and regurgitation of the valve in patients with right atrial isomerism. We examined seven consecutive patients with documented right atrial isomerism who subsequently underwent postmortem examination during a 10-year period. The degree of regurgitation and the diameters of the common valve were evaluated via cineangiography, and the site of regurgitation was evaluated by echocardiography. The morphology of the common atrioventricular valve was assessed further at autopsy. Cineangiography revealed valve diameters ranging from 14.8 to 27.8 mm (mean 20.9 mm). Valvar regurgitation was revealed within 2 months of birth in all patients. Regurgitation abruptly worsened in three patients after placement of a Blalock-Taussig shunt or a central shunt and postintubation. Autopsies revealed that the common atrioventricular valve consisted of four leaflets in five patients, and three leaflets in two. The anterior leaflets were large and protruding in all patients, and the lateral leaflets were thickened in six. All patients had a mass consisting of the left lateral leaflets and chordae with direct attachment of the chordae to the ventricular muscle (the right lateral leaflet was attached to the ventricular muscle and immobile in one patient). The lateral leaflets clung to the ventricular wall and exhibited poor movement in six patients. Leaflets with poor mobility corresponded to the regurgitant valvar site as assessed by echocardiography in six patients; and the regurgitation in three patients with acute deterioration occurred at the valvar side with poor mobility. It is concluded that the common atrioventricular valve in patients with right atrial isomerism has morphologic characteristics that may be associated with valvar regurgitation and malignant potential for abrupt deterioration after replacement of systemic-pulmonary shunting.
Atrioventricular septal defects: cross-sectional echocardiographic and morphologic comparisons
International Journal of Cardiology, 1986
We examined the cross-sectional echocardiographic findings of 171 patients with atrioventricular septal defects. The echocardiographic findings were confirmed by angiography, surgery and/or autopsy. The echocardiographic findings determined whether the common atrioventricular junction was guarded by a common valve or separate right and left valves. In addition, we were able to judge whether the bridging leaflets were related to the septal structures so as to permit both interatrial and interventricular communications [ 127 cases1 or whether the interatrial communication ("ostium primum atrial septal defect") [43 cases] or an interventricular communication [l case] existed in isolation. Defects existing with a common atrioventricular valve could be further classified as having minimal bridging of the antero-superior leaflet (Rastelli Type A (113 cases]); intermediate bridging (Rastelli Type B 13 cases]); or extreme bridging (Rastelli Type C [ 11 cases]). Of the patients with Down's syndrome, 9 had separate right and left valves while 66 had a common valve, all the latter existing in the setting of minimal bridging of the antero-superior leaflet. In the overall group, there were 9 cases having an unbalanced ventricular mass, 5 with right ventricular dominance and 4 with left dominance. Other associated defects were common. The echocardiographic findings were supplemented by pulsed Doppler examination. Atrioventricular valve insufficiency, when mild, was frequently demonstrated only in the right atrium just above the leaflets of the atrioventricular valve.
Is there another type of biventricular atrioventricular connection?
PubMed, 1999
In evaluating a 9-year-old girl, we encountered the following cardiac anomalies: a left atrioventricular valve (the morphologic left atrium on the left side, connected with the morphologic left ventricle); concordant atrioventricular connections; a right atrioventricular valve (a morphologic right atrium on the right side, connected with the morphologic right ventricle); concordant atrioventricular connection; a "central" atrioventricular valve (separating the morphologic right atrium from the morphologic left ventricle); and discordant atrioventricular connection with intact interatrial and interventricular septa. A right-to-left shunt passed through the central valvular structure, which was situated at the atrioventricular septum. This was the only means of mixing pulmonary and systemic blood. We suggest that this case presents a newly recognized cardiac anomaly.