Special Needs – Double Outlet Right Ventricle

Special Needs Guide

Double Outlet Right Ventricle (DORV); Taussig-Bing Anomaly; DORV with double-committed VSD; DORV with noncommitted VSD; DORV with subaortic VSD:

Normally the aorta arises from the left ventricle (the chamber of the heart that usually pumps blood to the body). The pulmonary artery normally arises from the right ventricle. With Double Outlet Right Ventricle, both arteries arise from the right ventricle. This is a problem because the right ventricle carries oxygen-poor blood, which the aorta then carries throughout the body. DORV always includes a ventricular septal defect (VSD). Pulmonary valve stenosis, or transposition of the great arteries may also be part of the defect. Children with DORV may have shortness of breath, fatigue, swelling of the legs, a bluish skin color, or excessive sweating.

Causes: Congenital heart defects are generally caused by an error in heart development in utero. The exact cause is unknown, although could be attributed to genetic or environmental factors. Rubella or drug and alcohol use during pregnancy increase the risks that a baby will be born with CHD.

Possible complications: Complications from DORV may include Congestive Heart Failure, pulmonary hypertension, and irreversible damage to the lungs.

Treatment: DORV requires open-heart surgery. The type of surgery depends on the individual child’s condition. A patch may be used to create a tunnel between the right and left ventricles. This allows the oxygenated blood to be moved to the correct ventricle. In more extensive procedures, surgeons may detach and reattach the aorta to the correct ventricle. In more serious cases, either ventricle may be too small or malformed to ever function correctly. Surgeons will reconfigure the heart and circulatory system so that the heart functions with one ventricle (pumping chamber), instead of two. This is called a Fontan procedure and and often requires three open heart surgeries. Many children adopted internationally will not have early intervention, which puts them at greater risk for additional complications.

Prognosis: The prognosis depends on the severity of the defects as well as the presence of other defects in the heart. Most children do not require continuous intervention and lead healthy lives.

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 [1] Love Without Boundaries – http://www.adoptspecialneeds.org/