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Further clarification on the variants of double-outlet right atrium.

ABSTRACT: Uniatrial but biventricular atrioventricular connection is a rare congenital cardiac abnormality where the left atria-ventricular connection is absent and the right atrio-ventricular connection straddles the crest of the muscular ventricular septum. This anomaly has been referred to as double outlet right atrium and the atrio-ventricular valve as a common atrioventricular valve in the past. In the absence of a primary atrial septal defect, the atrio-ventricular junction is not a common junction and the valve cannot hence be described as a common trio-ventricular valve. We demonstrate this paradox by an illustrative case and contrast this with two other cases where a common atrio-ventricular valve was predominantly committed to the right atrium.

SUBMITTER: Krishna MR

PROVIDER: S-EPMC9075570 | biostudies-literature | 2021 Oct-Dec

REPOSITORIES: biostudies-literature

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Further clarification on the variants of double-outlet right atrium.

Krishna Mani Ram MR Anderson Robert H RH

Annals of pediatric cardiology 20211001 4

Uniatrial but biventricular atrioventricular connection is a rare congenital cardiac abnormality where the left atria-ventricular connection is absent and the right atrio-ventricular connection straddles the crest of the muscular ventricular septum. This anomaly has been referred to as double outlet right atrium and the atrio-ventricular valve as a common atrioventricular valve in the past. In the absence of a primary atrial septal defect, the atrio-ventricular junction is not a common junction ...[more]

PMID: 35527746

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Project description:BackgroundDouble outlet right ventricle (DORV) describes a group of congenital heart defects where pulmonary artery and aorta originate completely or predominantly from the right ventricle. The individual anatomy of DORV patients varies widely with multiple subtypes classified. Although the majority of morphologies is suitable for biventricular repair (BVR), complex DORV anatomy can render univentricular palliation (UVP) the only option. Thus, patient-specific decision-making is critical for optimal surgical treatment planning. The evolution of image processing and rapid prototyping techniques facilitate the generation of detailed virtual and physical 3D models of the patient-specific anatomy which can support this important decision process within the Heart Team.Materilas and methodsThe individual cardiovascular anatomy of nine patients with complex DORV, in whom surgical decision-making was not straightforward, was reconstructed from either computed tomography or magnetic resonance imaging data. 3D reconstructions were used to characterize the morphologic details of DORV, such as size and location of the ventricular septal defect (VSD), atrioventricular valve size, ventricular volumes, relationship between the great arteries and their spatial relation to the VSD, outflow tract obstructions, coronary artery anatomy, etc. Additionally, physical models were generated. Virtual and physical models were used in the preoperative assessment to determine surgical treatment strategy, either BVR vs. UVP.ResultsMedian age at operation was 13.2 months (IQR: 9.6-24.0). The DORV transposition subtype was present in six patients, three patients had a DORV-ventricular septal defect subtype. Patient-specific reconstruction was feasible for all patients despite heterogeneous image quality. Complex BVR was feasible in 5/9 patients (55%). Reasons for unsuitability for BVR were AV valve chordae interfering with potential intraventricular baffle creation, ventricular hypoplasia and non-committed VSD morphology. Evaluation in particular of qualitative data from 3D models was considered to support comprehension of complex anatomy.ConclusionImage-based 3D reconstruction of patient-specific intracardiac anatomy provides valuable additional information supporting decision-making processes and surgical planning in complex cardiac malformations. Further prospective studies are required to fully appreciate the benefits of 3D technology.

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Further clarification on the variants of double-outlet right atrium.

Publications

Further clarification on the variants of double-outlet right atrium.

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