Tuesday, May 24, 2011

Can My Kid Really Do That?

Being a parent of a child with a heart defect is a frightening thing when you consider physical activity and the stress on the heart.  But summer is almost here; it's a time for swimming and baseball and lots of outdoor physical activity, which brings a discussion about exercise to mind.  Single ventricle kids have to work pretty hard sometimes to match what they want to do, with what their bodies allow them to do, but that is no reason to keep them out of the pool or off the baseball field. The heart is a muscle after all, and it needs exercise to stay conditioned. Research studies have shown that exercise can improve cardiac function in a child’s heart that has been damaged by a heart defect. Please talk with your child’s cardiologist about what is recommended for your child.  A general guideline for single ventricle kids is no competitive or contact sports due to the excessive strain on the heart and the risk of bleeding. There are however many other activities to enjoy; swimming, golf, tennis and bike-riding are great low impact aerobic activities.  Hydration is important in maintaining body temperature for single ventricle kids, so drinking a lot of water is a must when outdoors.

I recently read a wonderful story by an undergraduate student who wrote about her experience as a volunteer at a camp for heart kids.  Please follow this link to read her story:


The Hospital for Sick Children in Ontario Canada has an ongoing research study titled, “Optimizing Health among Children with Congenital Heart Defects,” which can be found at:


The warm summer months are when our children thrive, so get out there and enjoy it!  

Wednesday, May 11, 2011

What is Hypoplastic Left Heart Syndrome?

If you're visiting here, you are most likely not new to this diagnosis, but I thought it would be helpful to share what I learned when my son was first diagnosed with HLHS.

Hypoplastic left heart syndrome (HLHS) is a combination of several abnormalities of the heart and great blood vessels. It is a congenital (present at birth) syndrome, meaning that the heart defects occur due to abnormal underdevelopment of sections of the fetal heart during the first 8 weeks of pregnancy.

In the normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, and then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped out to the body through the aorta.
           
In hypoplastic left heart syndrome, also known as single ventricle condition, most of the structures on the left side of the heart are small and underdeveloped. The degree of underdevelopment differs from child to child. The structures affected usually include the following:
           
·         Mitral valve - the valve that controls blood flow between the left atrium and left ventricle in the heart.
·         Left ventricle - the lower left-hand chamber of the heart. It receives oxygen-rich (red) blood from the left atrium and pumps it into the aorta, which takes the blood to the body. The left ventricle must be strong and muscular in order to pump enough blood to the body to meet its requirements.
·         Aortic valve - the valve that regulates blood flow from the heart into the aorta.
·         Aorta - the largest artery in the body and the primary blood vessel leading from the heart to the body.
           
Perhaps the most critical defect in HLHS is the small, underdeveloped left ventricle. This chamber is normally very strong and muscular so it can pump blood to the body. When the chamber is small and poorly developed, it will not function effectively and cannot provide enough blood flow to meet the body's needs. For this reason, an infant with hypoplastic left heart syndrome will not live long without surgical intervention.
           
Hypoplastic left heart syndrome occurs in up to four out of every 10,000 live births. The syndrome comprises 8 percent of all cases of congenital heart disease. It is one of the top three heart abnormalities to cause problems in the newborn. HLHS occurs slightly more often in boys than in girls.
           
What causes hypoplastic left heart syndrome?
            Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. In hypoplastic left heart syndrome, there may be abnormalities of other organs, such as diaphragmatic hernia, omphalocele, and hypospadias.

In many children, HLHS occurs sporadically (by chance), with no clear reason evident for their development.
           
What are the symptoms of hypoplastic left heart syndrome?
            Infants with HLHS usually develop symptoms shortly after birth. The following are the most common symptoms of hypoplastic left heart syndrome. However, each child may experience symptoms differently. Symptoms may include:
           
·         Cyanosis (blue color of the skin, lips, and nail beds)
·         Pale skin
·         Sweaty or clammy skin
·         Cool skin
·         Heavy and/or rapid breathing
·         Fast heart rate
           
The symptoms of hypoplastic left heart syndrome may resemble other medical conditions and heart problems. Always consult your child's physician for a diagnosis.
           
How is hypoplastic left heart syndrome diagnosed?
            Your child's physician may have heard a heart murmur during a physical examination, and referred your child to a pediatric cardiologist for a diagnosis. A heart murmur is simply a noise caused by the turbulence of blood flowing through the obstruction from the right ventricle to the pulmonary artery. Symptoms your child exhibits will also help with the diagnosis.

A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and make other observations that help in the diagnosis. However, other tests are needed to help with the diagnosis.      

Chest x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
Electrocardiogram (ECG or EKG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage.
Echocardiogram (echo) - a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.
           
Treatment for hypoplastic left heart syndrome:
            Specific treatment for hypoplastic left heart syndrome will be determined by your child's physician based on:
           
·         Your child's age, overall health, and medical history.
·         Extent of the disease.
·         Your child's tolerance for specific medications, procedures, or therapies.
·         Expectations for the course of the disease.
·         Your opinion or preference.
           
Your child will most likely be admitted to the intensive care unit (ICU) or special care nursery once symptoms are noted. Initially, your child may be placed on oxygen, and possibly even on a ventilator, to assist his/her breathing. Intravenous (IV) medications may be given to help the heart and lungs function more efficiently.

There are two approaches offered to treat HLHS. Your child's cardiologist and cardiac surgeon will explain the risks and benefits to you. The options include the following:
           
·         A series of three operations that are done in stages: one shortly after birth, the second at about 6 months of age, and the final at about 18 months of age (these stages may vary). In this series of operations, the right ventricle is used as the main pumping chamber to the body, and blood flow is redirected to the lungs and the body with various surgical connections.

·         Heart transplantation.

Although we chose the route of surgical repairs over heart transplantation, I see the outcomes in the same way - our children are breaking the mold.  If you are interested in reading more about the treatment of HLHS at Children’s Hospital of Wisconsin, please follow the link: