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Updated: Aug 19, 2026

Murine Fetal Echocardiography
Published on: February 15, 2013
[Anatomy and embriology of double outlet right ventricle (author's transl)]
Insights
Double outlet right ventricle (DORV) exhibits diverse anatomical variations. This study proposes a classification based on great vessel relationships and ventricular septal defects, emphasizing detailed individual case descriptions due to DORV
Area of Science:
- Cardiovascular Anatomy
- Congenital Heart Disease Research
- Pathology
Context:
- Double outlet right ventricle (DORV) is a complex congenital heart defect.
- Understanding DORV's anatomical spectrum is crucial for diagnosis and treatment.
- Previous classifications may not fully capture DORV's heterogeneity.
Purpose:
- To define and classify anatomical variations of double outlet right ventricle (DORV).
- To analyze the relationship between great vessels and ventricular septal defects in DORV.
- To correlate autopsy findings with clinical data (angiographic and surgical).
Summary:
- Reviewed 64 autopsy cases of DORV, defining it as the origin of trunco-conal structures from the right ventricle.
- Identified diverse anatomical variations, including normally crossed, transposed, and side-by-side great arteries, and common truncus.
- Proposed a classification based on great vessel arrangement and ventricular septal defect presence/type, noting anterior VSDs in specific subtypes.
Impact:
- Highlights the significant heterogeneity of DORV, necessitating detailed individual anatomical assessment.
- Provides a framework for classifying DORV based on key anatomical features.
- Informs diagnostic strategies and surgical planning for patients with DORV.
Abstract:
After formulating the definition of double outlet right ventricle (DORV) as the persistence origin of the trunco-conal structures from the right ventricle, 64 autopsy cases meeting this definition were reviewed. A wide spectrum of anatomic variations of DORV were found. A classification is proposed based upon the type of relationship between the great vessels and upon the presence and type of ventricular septal defect. Twenty-five cases had normally crossed great arteries (13 of which had pulmonic stenosis), nine cases had transposed vessels and 23 cases had a double muscular conus and a side-by-side arrangement of the great vessels in the frontal view, a relationship called "partial distortion" or atypical transposition. Seven cases had absent trunco-conal septum (common truncus). Only one case had intact ventricular septum. Anterior ventricular septal defect was found only in cases with partial distortion or common truncus. Angiographic and surgical correlations are presented. Because of their great heterogeneity, the need for detailed description of the anatomy of the individual case of DORV is emphasized.
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