Definition

Single ventricle anomalies refer to a category of rare heart conditions that can develop in the 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 be present, as well.

Examples of single ventricle anomalies include:

  • 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
Heart Chambers and Valves
heart anatomy
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Causes

These anomalies are congenital defects. This means that the baby is born with the condition. The abnormality develops while the baby is forming in the womb. It is not known exactly why the heart develops this way in some babies.

Risk Factors

Specific risk factors for single ventricle anomalies are often unclear, but they may include:

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

Symptoms

Symptoms may include:

  • Blue or pale grayish skin color
  • Fast breathing
  • Fast heart rate
  • Sweating
  • Poor feeding/poor weight gain

A heart murmur may be detected during the exam.

Diagnosis

You will be asked about your child’s symptoms and medical history. A physical exam will be done.

Your child's bodily fluids may be tested. This can be done with blood tests.

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

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

Treatment

Talk with the doctor about the best treatment plan for your child. Often, surgery is needed right away. Treatment options include:

Medication

Medications may be given to keep blood flowing through the ductus arteriosus. The ductus arteriosus is a connection between the pulmonary artery and the aorta, which is the largest artery in the body. It usually closes within a few days after birth. Keeping this passage open is a temporary treatment. Other medications may be used as well.

Surgery

The goal of surgery is to:

  • Restore connections between the heart, lungs, and body
  • Improve circulation

Depending on the type of defect and how severe it is, there are several surgery options. For example, a shunt may be placed to restore connections between the heart and lungs, as well as the heart and the rest of the body. If there is too much blood flow, a band may be placed around the pulmonary artery. Fontan procedure is another option. This involves a series of surgeries with the goal being to reroute blood travel through the heart and lungs. In other cases, a heart transplant may be needed.

Lifelong Monitoring

Your child will have regular exams from a heart specialist. Your child may also need antibiotics prior to medical or dental procedures. This is to prevent an infection in the heart. Your child’s activity may also be limited.

Prevention

Preventing fetal heart defects may not always be possible, but you can reduce your risk with proper prenatal care.

Revision Information

  • Reviewer: Kari Kassir, MD
  • Review Date: 05/2014 -
  • Update Date: 06/03/2014 -
  • American Heart Association

    http://www.heart.org

  • Family Doctor—American Academy of Family Physicians

    http://familydoctor.org

  • Canadian Cardiovascular Society

    http://www.ccs.ca

  • Heart and Stroke Foundation of Canada

    http://ww2.heartandstroke.ca

  • Single ventricle anomalies and Fontan circulation. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/info/heart/diagnose/sv.htm. Updated December 2013. Accessed June 3, 2014.

  • Single ventricle defects. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects%5FUCM%5F307037%5FArticle.jsp. Updated March 26, 2014. Accessed June 3, 2014.

  • Single ventricle heart defects. Children’s Hospital of Philadelphia website. Available at: http://www.chop.edu/service/cardiac-center/heart-conditions/single-ventricle.html#treatment. Updated September 2013. Accessed June 3, 2014.