Double-outlet right ventricle (DORV)

Diagnosis

After birth, a healthcare provider might suspect a congenital heart defect if a child experiences growth delays or exhibits changes in lip, tongue, or fingernail color.

The provider may also detect a heart murmur during a stethoscope examination. Most heart murmurs are harmless and not indicative of a heart defect. However, some murmurs result from abnormal blood flow patterns in the heart.

Diagnostic tests for congenital heart defects include:

• Pulse oximetry: A fingertip sensor measures blood oxygen levels. Low oxygen levels may suggest heart or lung issues.
• Electrocardiogram (ECG or EKG): This noninvasive test records the heart’s electrical activity using chest electrodes. It helps identify irregular heart rhythms (arrhythmias).
• Echocardiogram: Sound waves (ultrasound) produce moving images of the heart, showing blood flow and valve function. When performed before birth, it’s called a fetal echocardiogram.
• Chest X-ray: This reveals heart and lung conditions, including heart enlargement or signs of heart failure, such as fluid in the lungs.
• Cardiac catheterization: A thin tube (catheter) inserted into a blood vessel, often in the groin, is guided to the heart. This provides detailed information on blood flow and heart function and allows for certain heart treatments.
• Heart magnetic resonance imaging (MRI): Used in adolescents and adults, it creates 3D images of the heart for precise measurement and evaluation of congenital heart defects.

Treatment

Most babies born with DORV (Double Outlet Right Ventricle) typically require open-heart surgery during their first year of life. Your healthcare provider will help you decide on the best course of action for surgery by considering:

• Any other heart or organ issues your baby may have.
• The overall health of your baby.
• The specific type of DORV your baby has.

The surgeon may choose from these approaches:

• Biventricular repair: If both ventricles are healthy and the DORV is not complex, the surgeon may recommend this option. It involves moving the aorta to the left ventricle.
• Intraventricular repair: In this procedure, the surgeon creates a tunnel through the VSD (Ventricular Septal Defect) to connect the aorta to the left ventricle. This tunnel is called a baffle.
• Univentricular repair: For more complex cases of DORV, the surgeon may suggest this approach, which redirects blood flow from the lower body directly to the lungs.

These surgical options are chosen based on the specific condition of your baby’s heart and overall health. Your healthcare team will guide you in making the best decision for your baby’s well-being.