- Double outlet right ventricle
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Double outlet right ventricle Classification and external resources ICD-10 Q20.1 ICD-9 745.11 OMIM 217095 DiseasesDB 32215 eMedicine ped/2509 ped/2508 MeSH D004310 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.
Anatomy and pathophysiology
DORV occurs in a multiple forms, with variability of great artery position and size, as well as of ventricular septal defect (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:
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- 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 Fallot
- 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
- but if there is pulmonic stenosis in addition, physiology resembles Tetralogy of Fallot
- 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 stenosis, physiology resembles Tetralogy of Fallot
External links
- Pediatric Heart Surgery
- The Congenital Heart Surgery Video Project
- Ashley: Repair of DORV
- synd/2285 at Who Named It?
- Illustrations at rch.org.au
Congenital heart defects (Q20–Q24, 745–746) Cardiac shunt/
heart septal defectAortopulmonary septal defectR→L: Double outlet right ventricle (Taussig–Bing syndrome) · Transposition of the great vessels (dextro, levo) · Persistent truncus arteriosusL→R: Sinus venosus atrial septal defect · Lutembacher's syndromeL→R and R→L: Eisenmenger's syndromeR→L, with other conditions: Tetralogy of FallotL→R: Ostium primumValvular heart disease/
heart chambersRightpulmonary valves (stenosis, insufficiency) · tricuspid valves (stenosis, atresia, Ebstein's anomaly) · Hypoplastic right heart syndrome (Uhl anomaly)LeftOther Dextrocardia · Levocardia · Cor triatriatum · Crisscross heart · Brugada syndrome · Coronary artery anomaly · Anomalous aortic origin of a coronary artery · Ventricular inversionCategories:- Congenital heart disease
- Disease stubs
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