Double outlet right ventricle

From Wikipedia, the free encyclopedia

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).

[edit] 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:

• 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

[edit] External links

• synd/2285 at Who Named It
• Illustrations at rch.org.au

v • d • e Congenital malformations and deformations of circulatory system (Q20-Q28, 745-747) Cardiac chambers and connections Persistent truncus arteriosus - Double outlet right ventricle (Taussig-Bing syndrome) - Transposition of the great vessels (dextro, levo) Cardiac septa Ventricular septal defect - Atrial septal defect (Lutembacher's syndrome) - Atrioventricular septal defect (Ostium primum) - Tetralogy of Fallot - Eisenmenger's syndrome Valvular heart disease/ heart chambers Right: pulmonary and tricuspid valves pulmonary valves (stenosis, insufficiency) - tricuspid valves (stenosis, atresia) - Ebstein's anomaly Left: aortic and mitral valves aortic valves (stenosis, insufficiency, bicuspid) - mitral valves (stenosis, regurgitation) - Hypoplastic left heart syndrome Other congenital malformations of heart Dextrocardia - Levocardia - Cor triatriatum Vascular malformation Great arteries aorta: Patent ductus arteriosus - Aortic coarctation - Interrupted aortic arch - Overriding aorta - Aneurysm of sinus of Valsalva - Vascular ring pulmonary artery: Pulmonary atresia Great veins superior vena cava: Persistent left superior vena cava pulmonary vein: Total anomalous pulmonary venous connection - Scimitar syndrome Other Arteriovenous malformation (Cerebral arteriovenous malformation) See also non-congenital conditions (I, 390-459)