Double-Outlet Right Ventricle (DORV)

Double-outlet right ventricle (DORV) is a rare congenital heart defect where both the aorta and pulmonary artery arise from the right ventricle, instead of the normal arrangement where the aorta arises from the left ventricle and the pulmonary artery arises from the right ventricle.

Causes and Risk Factors

DORV is a congenital heart defect, meaning it is present at birth. The exact cause of DORV is not known, but it is thought to occur during fetal development when the heart is forming. Certain genetic conditions, such as trisomy 13 or trisomy 18, can increase the risk of developing DORV.

Types of DORV

There are several types of DORV, including:

• Subaortic type: The aorta is located below the pulmonary valve and arises from the right ventricle.
• Subpulmonary type: The pulmonary artery is located below the aortic valve and arises from the right ventricle.
• Taussig-Bing type: Both the aorta and pulmonary artery arise from the right ventricle, but the aorta is located anterior to the pulmonary artery.
• Non-committed type: The aorta and pulmonary artery arise from the right ventricle, but are not committed to either the subaortic or subpulmonary position.

Symptoms

The symptoms of DORV can vary depending on the severity of the defect and the presence of other heart defects. Common symptoms include:

• Cyanosis (blue discoloration of the skin)
• Shortness of breath
• Fatigue
• Poor feeding in infants
• Sweating
• Pale skin

Diagnosis

DORV is typically diagnosed using a combination of the following tests:

• Echocardiogram: An ultrasound test that uses sound waves to create images of the heart.
• Chest X-ray: A test that uses radiation to create images of the heart and lungs.
• Electrocardiogram (ECG): A test that measures the electrical activity of the heart.
• Cardiac catheterization: A test that uses a small tube to inject dye into the heart and measure blood pressure and oxygen levels.

Treatment

The treatment for DORV typically involves surgery, which may include:

• Arterial switch operation: A procedure that switches the positions of the aorta and pulmonary artery.
• Rastelli procedure: A procedure that creates a tunnel between the left ventricle and the aorta, allowing blood to flow from the left ventricle to the aorta.
• Fontan procedure: A procedure that redirects blood flow from the right ventricle to the pulmonary artery, bypassing the right ventricle.

Prognosis

The prognosis for DORV depends on the severity of the defect and the presence of other heart defects. With surgical treatment, most children with DORV can lead active and normal lives. However, they may require ongoing medical care and monitoring to prevent complications.