Double outlet right ventricle
Encyclopedia
Double outlet right ventricle (DORV) is a form of congenital heart disease where both of the great arteries
connect (in whole or in part) to the right ventricle
(RV). In some cases it is found that this occurs on the left side of the heart rather then the right side.
(VSD) location. It can occur with or without transposition of the great arteries. The clinical manifestations are similarly variable, depending on how the anatomical defects affect the physiology
of the heart, in terms of altering the normal flow of blood from the RV and left ventricle
(LV) to the aorta
and pulmonary artery
. For example:
Great arteries
Great arteries is a term used to refer collectively to the primary arteries of the heart, which include:* Pulmonary artery: the vessel that carries oxygen-depleted blood from the right ventricle to the lungs....
connect (in whole or in part) to the right ventricle
Right ventricle
The right ventricle is one of four chambers in the human heart. It receives deoxygenated blood from the right atrium via the tricuspid valve, and pumps it into the pulmonary artery via the pulmonary valve and pulmonary trunk....
(RV). In some cases it is found that this occurs on the left side of the heart rather then the right side.
Anatomy and pathophysiology
DORV occurs in a multiple forms, with variability of great artery position and size, as well as of ventricular septal defectVentricular septal defect
A ventricular septal defect is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart.The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes.The membranous...
(VSD) location. It can occur with or without transposition of the great arteries. The clinical manifestations are similarly variable, depending on how the anatomical defects affect the physiology
Physiology
Physiology is the science of the function of living systems. This includes how organisms, organ systems, organs, cells, and bio-molecules carry out the chemical or physical functions that exist in a living system. The highest honor awarded in physiology is the Nobel Prize in Physiology or...
of the heart, in terms of altering the normal flow of blood from the RV and left ventricle
Left ventricle
The left ventricle is one of four chambers in the human heart. It receives oxygenated blood from the left atrium via the mitral valve, and pumps it into the aorta via the aortic valve.-Shape:...
(LV) to the aorta
Aorta
The aorta is the largest artery in the body, originating from the left ventricle of the heart and extending down to the abdomen, where it branches off into two smaller arteries...
and pulmonary artery
Pulmonary artery
The pulmonary arteries carry deoxygenated blood from the heart to the lungs. They are the only arteries that carry deoxygenated blood....
. 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 Tetralogy of FallotTetralogy of FallotTetralogy of Fallot is a congenital heart defect which is classically understood to involve four anatomical abnormalities...
- in DORV with a subpulmonic VSD (called Taussig-Bing syndromeTaussig-Bing syndromeTaussig-Bing syndrome is a cyanotic congenital heart defect in which the patient has both double outlet right ventricle and subpulmonic ventricular septal defect ....
), 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- but if there is pulmonic stenosisPulmonic stenosisPulmonic stenosis, also known as Pulmonary stenosis, is a dynamic or fixed obstruction to flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood....
in addition, physiology resembles Tetralogy of FallotTetralogy of FallotTetralogy of Fallot is a congenital heart defect which is classically understood to involve four anatomical abnormalities...
- but if there is pulmonic stenosis
- 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 pulmonic stenosisPulmonic stenosisPulmonic stenosis, also known as Pulmonary stenosis, is a dynamic or fixed obstruction to flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood....
, physiology resembles Tetralogy of FallotTetralogy of FallotTetralogy of Fallot is a congenital heart defect which is classically understood to involve four anatomical abnormalities...
- but again, if there is pulmonic stenosis