Definition

Single ventricle anomalies are rare heart conditions that can happen in a growing fetus. In a normal heart, the two ventricles work by collecting blood and pumping it to the lungs or the rest of the body. With this condition, one of the ventricles does not develop properly. The defect can be mild to severe. Other heart problems may also be present.

Examples of single ventricle anomalies are:

• Tricuspid atresia—tricuspid valve does not develop
• Pulmonary atresia—pulmonary valve does not develop
• Hypoplastic left heart syndrome—left side of the heart does not develop properly
• Double inlet left ventricle—large left ventricle and small right ventricle

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Causes

These problems are caused by congenital defects. This means that the problem develops in the womb and a baby is born with it. It is not known exactly why this happens.

Risk Factors

Specific risk factors are often unclear, but they may be:

• A family history of congenital heart defect
• Other heart defects
• Certain chromosomal disorders
• Environmental exposure to chemicals that cause birth defects

Symptoms

Problems may be:

• Blue or pale grayish skin color
• Fast breathing
• Rapid heartbeat
• Sweating
• Poor feeding
• Poor weight gain

Diagnosis

The doctor will ask about your child’s symptoms and health history. A physical exam will be done. It will focus on the heart. This problem may be suspected if the doctor hears a heart murmur.

Blood tests will be done.

Images may be taken of your child's body. This can be done with:

• Echocardiogram
• Chest x-ray
• MRI scan
• CT scan

Your child's heart function may be tested. This can be done with:

• Electrocardiogram
• Cardiac catheterization

Treatment

The goal of treatment is to improve heart function and blood flow. Choices are:

• Medicines to keep blood flowing through a connection that normally closes a few days after birth
• Surgery to:
  • Place a shunt to restore connections between the heart and lungs as well as the heart and rest of the body
  • Place a band around the pulmonary artery if there is too much blood flow
  • Reroute blood flow through the heart and lungs through a series of surgeries

Children with severe problems may need a heart transplant.

Lifelong heart monitoring will be needed after treatment.

Prevention

There are no known guidelines to prevent this heart problem.