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Congenital Defects Tutorial
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Septal Defects Right Heart Lesions Left Heart Lesions Anomalies of Arteries and Veins
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.