Definition

Double-outlet right ventricle (DORV) is a rare heart defect. The aorta, which is the largest artery in the body, comes from the heart’s left ventricle. With DORV, the aorta and the pulmonary artery are attached to the right ventricle.

A heart defect called ventricular septal defect (VSD) usually happens with DORV. VSD is a hole in the wall between the right and left ventricle. DORV can be categorized based on the position of the VSD.

Along with these problems, the pulmonary valve may be narrowed. DORV is a serious health problem that needs care right away.

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Causes

DORV is present at birth. It is not known exactly why the heart does not form the right way.

Risk Factors

For many heart defects, the risk factors are not clear. Some factors for DORV may be:

• Family history of congenital heart defects
• Certain chromosomal problems

Symptoms

Symptoms may vary based on where the VSD is. Your child may have:

• Bluish skin color
• Poor feeding/slow weight gain
• Lack of energy
• Sweating
• Rapid breathing
• Shortness of breath

Diagnosis

You will be asked about your child’s symptoms and health history. A physical exam will be done. During the exam, the doctor may also find a heart murmur.

Pictures may be taken of your child's bodily structures. This can be done with:

• Chest x-ray
• Echocardiogram
• MRI scan
• Cardiac catheterization

Your child's heart activity may be measured. This can be done with electrocardiogram (EKG).

Treatment

Talk with the doctor about the best plan for your child. Often, surgery is needed right away. Your child’s options are:

Prevention

DORV can’t be prevented because the cause is not known.