(HLHS)
Definition
Definition
Hypoplastic left heart syndrome is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood returns to the left atrium and goes into the left ventricle. The blood then moves out to the rest of the body.
With this syndrome, structures on the left side of the heart, which includes the aorta, aortic valve, left ventricle, and mitral valve, may be:
- Too small
- Absent
- Abnormally developed
Since the heart cannot function properly, oxygen-rich blood flow to the body is limited. This condition requires immediate care from a doctor.
Heart Chambers and ValvesCopyright © Nucleus Medical Media, Inc.
|
Blood Flow Through the HeartCopyright © Nucleus Medical Media, Inc.
|
Causes
Causes
Hypoplastic left heart syndrome is present at birth. It is not known exactly why the heart does not develop normally.
Risk Factors
Risk Factors
These factors increase your chance of having a child with hypoplastic left heart syndrome:
- Previous pregnancy with fetal heart abnormalities or fetal loss
- Family history of congenital heart defects
Symptoms
Symptoms
Symptoms usually appear within days after birth. Tell the doctor if you notice the following in your infant or child:
- Blue/gray skin color
- Cool skin
- Rapid or difficult breathing
- High heart rate
- Sweaty, clammy skin
- Poor feeding
Diagnosis
Diagnosis
You will be asked about your child’s symptoms and medical history. A physical exam will be done.
Images may be taken of your child’s chest. This can be done with:
Electrocardiogram (EKG) can monitor the heart’s electrical activity.
Treatment
Treatment
Talk with your doctor about the best treatment plan for your child. Some defects may be so severe that they are difficult to treat. Treatment options include:
Medications
Medications are necessary 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
Surgery may be done to improve blood flow. This can be done through a variety of reconstructive and shunting procedures. Surgeries are usually done in stages:
- After birth
- 4-6 months of age
- 2-4 years of age
Lifelong Monitoring
Your child will need to see a heart specialist regularly. Heart medication will be needed throughout your child’s life.
Prevention
Prevention
There are no current guidelines to prevent hypoplastic left heart syndrome because the cause is unknown. Getting proper prenatal care is always important.
RESOURCES:
Family Doctor—American Academy of Family Physicians http://familydoctor.org
CANADIAN RESOURCES:
Heart and Stroke Foundation of Canada http://www.heartandstroke.ca
References:
http://www.ebscohost.com/dynamed
Updated January 20, 2014. Accessed June 3, 2014.
Hypoplastic left heart syndrome. Johns Hopkins Children’s Center website. Available at:
http://www.hopkinschildrens.org/Hypoplastic-Left-Heart-Syndrome.aspx
Updated May 16, 2011. Accessed June 3, 2014.
Hypoplastic left heart syndrome (HLHS) in children. Boston Children’s Hospital website. Available at:
http://www.childrenshospital.org/conditions-and-treatments/conditions/hypoplastic-left-heart-syndrome-hlhs
Accessed June 3, 2014.
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 March 26, 2014. Accessed June 3, 2014.
Last Updated: 6/4/2014