Double Inlet Ventricle (DIV)

Double inlet ventricle (DIV) is a rare congenital heart defect (CHD) that changes the anatomy of the heart’s four chambers and affects circulation. Instead of having two working ventricles, a child with DIV has only one working ventricle. Depending on how DIV develops in a child, it may require treatment immediately after birth. That’s why it’s important for a newborn who hasn’t been diagnosed prenatally but has symptoms of one of the two types of DIV — double inlet left ventricle or double inlet right ventricle — and hasn’t been diagnosed prenatally to promptly have their circulation and heart assessed.

DIV can be treated through cardiac catheterization and cardiac surgery. The most common surgical treatment strategy is single ventricle palliation, which gives children a Fontan, or single ventricle, circulation. Children who rely on a single-ventricle heart can receive lifetime support. An alternative surgical treatment option that was pioneered by Boston Children’s, ventricular septation, can benefit some children who rely on their left ventricle.

Typically, the left atrium is connected to the left ventricle, while the right atrium is connected to the right ventricle. With DIV, both atria are connected to the one working ventricle.

Children are born with DIV. It is believed to happen early in pregnancy, when the heart is developing, but the exact cause is unknown.

A routine prenatal ultrasound during pregnancy may detect DIV, prompting a secondary test, a fetal echocardiogram. If your child shows symptoms of DIV after birth, a cardiologist will first listen to their heart and lungs and measure their oxygen level. This exam can give your cardiologist an idea of the condition.

The cardiologist may also request an:

• Electrocardiogram (ECG or EKG)
• Echocardiogram (cardiac ultrasound)
• Chest X-ray
• Cardiac catheterization

If your child has DILV, one of their two “great” arteries (either the pulmonary artery or the aorta) has to connect to the left ventricle through a side chamber of the heart called the bulboventricular foramen (BVF). How urgently they will need care depends on which of the two arteries uses the BVF for that connection.

• When the aorta comes from the left ventricle and the pulmonary artery comes from the BVF, your child shouldn’t need treatment immediately after birth.
• When the aorta comes from the BVF that means your child’s arteries are switched (the condition TGA). They could also have coarctation of the aorta (a narrowed aorta). If your child has these conditions, their circulation is not optimal. They may need to have surgery almost immediately after birth.

• Ventricular septal defect (VSD), a hole in the heart wall that separates the ventricles
• Coarctation of the aorta
• Pulmonary atresia, when the pulmonary valve doesn’t develop properly
• Pulmonary valve stenosis, a narrowing in the opening of the pulmonary valve
• Leakage of the atrioventricular valves
• Poor contraction of the heart muscle

Almost all children who have DIV need surgical treatment. The timing depends on the severity. Some newborns can wait a few months. Others need treatment immediately after birth because they are at risk. Here are treatment options our Department of Cardiac Surgery will consider for those children:

Immediate treatment options for some newborns

• If your child has too little blood flow reaching their lungs, their blood-oxygen saturations can be monitored carefully. But if your child has extreme cyanosis or hypoxia (oxygen deficiency), they may need either a stent or shunt to improve blood flow.
• If there is too much blood flow going to their lungs, they may need either a pulmonary band or pulmonary flow restrictor to lessen blood flow.
• If your child has TGA, coarctation of the aorta, or hypoplastic aortic arch along with DILV, there are several surgical options that depend on the BVF. They can have aortic arch reconstruction and pulmonary arterial banding if the BVF can support circulation. If the BVF cannot support circulation, they can have either an “arterial switch” operation or a procedure known as Damus-Kaye-Stansel (DKS).
• If your child’s circulation is stable, specialists from our Single Ventricle Program will monitor your child until they are ready for surgery.

Our Cardiac Surgery team achieves some of the best outcomes in the world, but we’re also known for our compassionate, family-centered approach to care. We will provide your family with the information, resources, and support you will need before, during, and after your child’s treatment.