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Total Anomalous Pulmonary Venous Connection Coarctication of the Aorta Patent Ductus Arteriosus Interrupted Aortic Arch Vascular Rings and Slings D-Transposition of Great Arteries Congenitally "Corrected" Transposition of Great Arteries Double Outlet Right Ventricle

Double Outlet Right Ventricle

  • The term double outlet right ventricle refers to a defect where both great arteries arise above the right ventricle.
  • In most cases of double outlet right ventricle the great vessels are found lying side by side in a d-transposition configuration with the aorta on the right and pulmonary artery on the left.
  • Rather than its own single anomaly, it is associated with other cardiac abnormalities. A ventricular septal defect is almost always present and 75% of patients have some degree of pulmonary stenosis. Transposition of the great, septal defects, or atrioventricular atresia, and a variety of valvular problems are also commonly seen with a double outlet right ventricle defect.
  • The hemodynamics involved with double outlet right ventricle are dependent on the anatomy of the great vessels and associated defects. Decreased arterial oxygen saturation is almost always present but as a result of wide variation in anatomy, patients may or may not appear cyanotic and/or develop congestive heart failure, or be asymptomatic.
  • Management of double outlet right ventricle depends on associated defects and the type of surgical correction they are amenable to.