Double outlet right ventricle

Double outlet right ventricle
Classification and external resources
Specialty medical genetics
ICD-10 Q20.1
ICD-9-CM 745.11
OMIM 217095
DiseasesDB 32215
MedlinePlus 007328
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 than the right side.

Anatomy and pathophysiology

DORV occurs in 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:

Treatment

DORV is treated with surgery.

Epidemiology

DORV affects between 1% and 3% of people born with congenital heart defects.[1]

Chromosomal abnormalities were reported in about 40% of reported cases in the medical literature.[1]

References

  1. 1 2 Obler D, Juraszek AL, Smoot LB, Natowicz MR (August 2008). "Double outlet right ventricle: aetiologies and associations". J. Med. Genet. 45 (8): 481–97. doi:10.1136/jmg.2008.057984. PMID 18456715.
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