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Double outlet right ventricle


Alternative Names:

DORV; Taussig-Bing anomaly; DORV with doubly-committed VSD; DORV with noncommitted VSD; DORV with subaortic VSD

Symptoms:

Symptoms of DORV may include:

  • Baby tires easily, especially when feeding
  • Bluish color of the skin and lips
  • Clubbing (thickening of the nail beds) on toes and fingers (late sign)
  • Failure to gain weight and grow
  • Pale coloring
  • Sweating
  • Swollen legs or abdomen
  • Trouble breathing
Exams and Tests:

Signs of DORV may include:

  • Enlarged heart
  • Heart murmur
  • Rapid breathing
  • Rapid heartbeat

Tests to diagnose DORV include:

  • Chest x-rays
  • Passing a thin, flexible tube into the heart to measure blood pressure and inject dye for special pictures of the heart and arteries (cardiac catheterization)
  • Ultrasound exam of the heart (echocardiogram)
  • Using magnets to create images of the heart (MRI)
Treatment:

Treatment requires surgery to close the hole in the heart and direct blood from the left ventricle into the aorta. Surgery may also be needed to move the pulmonary artery or aorta.

Factors that determine the type and number of operations the baby needs include:

  • The type of DORV
  • The severity of the defect
  • The presence of other problems in the heart
  • The child's overall condition
Outlook (Prognosis):

How well the baby does depends on:

  • The size of the VSD
  • Its location
  • The size of the pumping chambers
  • The location of the aorta and pulmonary artery
  • The presence of other complications (such as coarctation of the aorta and mitral valve problems)
  • The baby's overall health at the time of diagnosis
  • Whether lung damage has occurred from too much blood flowing to the lungs for a long period of time
Possible Complications:

Complications from DORV may include:

  • Congestive heart failure (CHF)
  • High blood pressure in the lungs
  • Irreversible damage to the lungs due to untreated high blood pressure in the lungs

Children with this congenital heart disease may need to take antibiotics before dental treatment. This prevents infections around the heart. Antibiotics may also be needed after surgery.

When to Contact a Medical Professional:

Call your health care provider if your child seems to tire easily, has trouble breathing, or has bluish skin or lips. You should also consult your health care provider if your baby is not growing or gaining weight.

References:

Baldwin HS, Dees Ellen. Embryology and physiology of the cardiovascular system. In: Gleason CA, Devaskar S, eds. Avery's Diseases of the Newborn. 9th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 50.


Review Date: 5/14/2014
Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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