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
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.
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 may include:
Blue or pale grayish skin color
Poor feeding/poor weight gain
You will be asked about your child’s symptoms and medical history. A physical exam will be done.
Talk with the doctor about the best treatment plan for your child. Often, surgery is needed right away. Treatment options include:
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.
The goal of surgery is to:
Restore connections between the heart, lungs, and body
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
may be needed.
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.
Preventing fetal heart defects may not always be possible, but you can reduce your risk with proper prenatal care.
Single ventricle anomalies and Fontan circulation. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/info/heart/diagnose/sv.htm. Updated July 2016. Accessed December 27, 2017.
Single ventricle defects. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects_UCM_307037_Article.jsp. Updated September 12, 2017. Accessed December 27, 2017.
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. Accessed December 27, 2017.
Last reviewed November 2018 by
EBSCO Medical Review Board
Kari Kassir, MD
Last Updated: 12/20/2014
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