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Published on: May 13, 2021
Further morphological observations on hearts with twisted atrioventricular connections (criss-cross hearts)
1From the Department of Paediatrics, The National Heart and Lung Institute, London, United Kingdom.
Insights
Twisted atrioventricular connections feature a superiorly displaced tricuspid valve due to septal malalignment. This anatomical variation explains unique angiocardiographic findings, including atrial and ventricular recesses.
Area of Science:
- Cardiovascular Anatomy
- Congenital Heart Disease
- Medical Imaging
Background:
- Twisted atrioventricular connections are a rare congenital anomaly.
- Understanding the morphologic substrate is crucial for diagnosis.
Purpose of the Study:
- To identify the unifying morphologic feature of twisted atrioventricular connections.
- To correlate anatomical findings with angiocardiographic appearances.
Main Methods:
- Autopsy examination of seven hearts with twisted atrioventricular connections.
- Morphologic analysis of atrial and ventricular septal structures.
- Angiocardiographic correlation.
Main Results:
- A superior and anterior tricuspid valve position due to septal malalignment was the characteristic feature.
- Unusual recesses were observed in the right atrium, right ventricle, and left ventricle.
- Right ventricular hypoplasia was noted in cases with recess in the outflow tract.
Conclusions:
- Septal malalignment and tricuspid valve displacement define twisted atrioventricular connections.
- Morphologic findings explain the distinct angiocardiographic patterns.
- Further study of these anatomical variations is warranted.
Abstract:
A superior and anterior location of the tricuspid valve in the setting of malalignment between the atrial and ventricular septal structures was the characteristic unifying morphologic feature of seven autopsied hearts with twisted atrioventricular connections. The peculiar angiocardiographic appearances of this lesion could be readily explained by the findings of unusual recesses in the right atrium, in the right ventricle, and in the left ventricle. The recess in the morphologically left ventricle was seen in each case at the right posterior part (or, in the hearts with left-handed ventricles, at the left part) of the chamber. Displacement of the right-sided atrioventricular valve far from the inferior caval vein (vena cava) had produced the recess in the morphologically right atrium; this feature was most prominent in the heart in which the atrioventricular connections were discordant. The recess within the right ventricular outflow tract was seen in four hearts, in all of which the inlet and apical trabecular components of the right ventricle were hypoplastic.
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