Double outlet right ventricle (DORV) is a form of

congenital heart disease A congenital heart defect (CHD), also known as a congenital heart anomaly, congenital cardiovascular malformation, and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital he ...

where both of the

great arteries The great arteries are the primary arteries that carry blood away from the heart, which include: * ''Pulmonary artery'': the vessel that carries oxygen-depleted blood from the right ventricle to the lungs. * ''Aorta'': the blood vessel through whic ...

connect (in whole or in part) to the

right ventricle A ventricle is one of two large chambers located toward the bottom of the heart that collect and expel blood towards the peripheral beds within the body and lungs. The blood pumped by a ventricle is supplied by an atrium (heart), atrium, an adjace ...

(RV). In some cases it is found that this occurs on the left side of the heart rather than the right side.

Cause

Pathogenesis

DORV occurs in multiple forms, with variability of great artery position and size, as well as of

ventricular septal defect A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. It's a common heart problem present at birth ( congenital heart defect). The extent of the opening may vary ...

(VSD) location. It can occur with or without

transposition of the great arteries Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenit ...

. The clinical manifestations are similarly variable, depending on how the anatomical defects affect the

physiology Physiology (; ) is the science, scientific study of function (biology), functions and mechanism (biology), mechanisms in a life, living system. As a branches of science, subdiscipline of biology, physiology focuses on how organisms, organ syst ...

of the heart, in terms of altering the normal flow of blood from the RV and

left ventricle A ventricle is one of two large chambers located toward the bottom of the heart that collect and expel blood towards the peripheral beds within the body and lungs. The blood pumped by a ventricle is supplied by an atrium, an adjacent chamber in t ...

(LV) to the

aorta The aorta ( ; : aortas or aortae) is the main and largest artery in the human body, originating from the Ventricle (heart), left ventricle of the heart, branching upwards immediately after, and extending down to the abdomen, where it splits at ...

and

pulmonary artery A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the ''main pulmonary artery'' or ''pulmonary trunk'' from the heart, and ...

. For example: :*in DORV with a subaortic VSD, blood from the LV flows through the VSD to the aorta and blood from the RV flows mainly to the pulmonary artery, yielding physiology similar to

:*in DORV with a subpulmonic VSD (called Taussig-Bing syndrome), blood from the LV flows through the VSD to the pulmonary artery and blood from the RV flows mainly to the aorta, yielding physiology similar to

Transposition of the Great Arteries Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenit ...

:**but if there is

pulmonic stenosis Pulmonic stenosis, is a dynamic or fixed obstruction of flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood. Signs and symptoms Some individuals with mild PS may not experience any sympt ...

in addition, physiology resembles

Tetralogy of Fallot Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are: * Pulmonary stenosis, which is narrowing of the exit from the r ...

:*in other forms of DORV, blood from both ventricles is substantially mixed in the RV, yielding physiology that resembles a large VSD :**but again, if there is

, physiology resembles

Treatment

DORV is treated with surgery.

Epidemiology

DORV affects between 1% and 3% of people born with

congenital heart defect A congenital heart defect (CHD), also known as a congenital heart anomaly, congenital cardiovascular malformation, and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital h ...

s. Chromosomal abnormalities were reported in about 40% of reported cases in the medical literature.

References

External links

{{Congenital malformations and deformations of circulatory system Congenital heart defects