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Double outlet right ventricle
Authors: Doctor Jim Wilkinson 1
Creation date: July 2002
Updated: December 2003
February 2005
Scientific Editor: Professor Robert H Anderson
1

Department of cardiology Royal Children's Hospital Flemington Road, Parkville VIC 3052 Melbourne,
Australia. jim.wilkinson@rch.org.au
Abstract
Keywords
Disease name and synonyms
European Paediatric Cardiac CodeIntroduction
Differential diagnosis
Frequency
Clinical description
Etiology
Diagnostic methods
Prognosis
References
Abstract
The term "Double Outlet Right Ventricle" (DORV) refers to any cardiac anomaly in which both the aorta
and pulmonary trunk originate, predominantly or entirely, from the right ventricle. A wide spectrum of
malformations occurs. Some have features similar toFallot's Tetralogy, others resemble Transposition
with ventricular septal defect and many other variations occur, some simple and others complex. The
clinical picture varies widely, depending on the combination of abnormalities present. Cyanosis is
frequent, but not universal. Heart failure with breathlessness, poor feeding and slow weight gain is also
common. The malformation results from an errorin formation of the outlet part of the ventricular loop
during early embryonic life, probably within the first three to four weeks after conception (between 5 and 6
weeks gestational age). In most cases the defect is "sporadic" in pattern and there is no identifiable
genetic cause. The primary diagnostic tool is echocardiography. The overall frequency is not well
documented as the conditionoverlaps with a range of defects and is often not categorized solely as
DORV in epidemiological studies of congenital heart disease. In its many forms it is probably present in
3% or more of all congenital heart defects, thus indicating a birth incidence of less than 1:3,000. Most
affected patients can undergo surgical repair.
Keywords
ventricular septal defect, Taussig-Bing Malformation,Eisenmenger’s "Anomaly", Fontan procedure

Disease name and synonyms
- Double Outlet Right Ventricle (DORV)
- Taussig-Bing Malformation
- Tetralogy of Fallot with extreme dextroposition
of the aorta

- Eisenmenger’s "Anomaly"
Ventricular Septal Defect)

(Eisenmenger

European Paediatric Cardiac Code
Reference of Double Outlet Right Ventricle is
01.01.04.

1
Wilkinson J. Double outletright ventricle. Orphanet encyclopedia, February 2005.
http://www.orpha.net/data/patho/GB/uk-DORV05.pdf

Introduction
The term "Double Outlet Right Ventricle" refers
to any cardiac anomaly in which both the aorta
and pulmonary trunk originate, predominantly or
entirely, from the right ventricle (RV) (1). In this
situation, the left ventricle (LV) has no direct
outlet to either greatartery and ejects through an
interventricular communication, usually referred
to as a "ventricular septal defect" (VSD), (which
is almost invariably present) into the right
ventricle. Rarely there may be no "VSD" and the
LV is then extremely hypoplastic.
The literature on DORV is substantial, and
includes descriptions of a large range of
anatomic variations (2, 3) which are reflected in
manydifferent clinical presentations. The
medical and surgical management of this entity
is also heterogeneous and needs to be tailored
to the particular problems of each affected child
(4-7).
A logical and step-by-step approach to the
diagnosis and classification of DORV is
essential. Atrial arrangement (and venous
connections) must be ascertained, and the
atrioventricular connectionsestablished with
certainty. Identification of the way in which the
two great arteries arise from the right ventricle is
important, including their inter-relationships and
the nature and severity of any obstructive
problems. The size, site, and morphology of the
interventricular
communication,
which
is
categorised in a similar way to that used for
isolated ventricular septal defects, are also...