Posts Tagged ‘ hypertrophic cardiomyopathy ’

Children With Broken Hearts

Thursday, May 3rd, 2012

After reading the latest findings on sudden cardiac death, we asked our advisor Darshak Sanghavi, M.D., chief of the division of pediatric cardiology and associate professor of pediatrics at University of Massachusetts Medical School, to put this frightening problem into perspective and help parents understand the prevention steps they can take. Here’s what he had to say:

Earlier this year, the American Academy of Pediatrics pointed out that several thousand young people die each year of unexplained sudden cardiac death. These cases are deeply tragic, and though rare, the sudden loss of a previously healthy child often leads to a great deal of concern among all parents.

It turns out that children’s heart problems are very different than those in adults. Typically, adult problems result from long-standing damage to arteries, buildup of cholesterol, and other long-term problems that can lead to sudden blockages. Doctors refer to these as “myocardial infarctions,” more commonly known as heart attacks.

In children, the problem is very different. Children who die suddenly have hidden birth defects of the heart structure or electrical system. For example, the leading cause of sudden death in young athletes is a genetic disorder caused by faulty proteins called hypertrophic cardiomyopathy, which by some estimates affects roughly one in 500 people. Like weeds that overrun an unkempt yard, the heart muscle fibers proliferate rapidly and in a disorganized manner, often leading to a tripling or quadrupling in heart size during adolescence (see a picture here). People with HCM usually have no signs that this is happening until they’re exercising one day and the electrical system in the heart suddenly fails. The heart takes on the appearance of a bag of worms struggling to get free (a problem called ventricular fibrillation), and cardiac arrest occurs.

Other hidden problems, which can’t be detected by conventional check-ups in a doctor’s office with a stethoscope, include coronary artery defects (for example, a twisted or abnormal blood vessel) and electrical defects (one is called long QT syndrome).

So what is a parent to do?  To begin, it’s important not to worry too much. Again, sudden cardiac death in children is still extremely rare. But it’s useful to know how to advocate for your child and others who may have these kinds of hidden heart problems. Here is my advice:

  1. Schools and athletic facilities should have ready access to a device called an automated external defibrillator (AED). In the rare case of sudden cardiac arrest, seconds count. That’s why having AEDs in public places in proven to save lives and many locales now mandate having them in airports, health clubs, and some schools.
  2. Talk to your pediatrician if your child has any risk factors for sudden cardiac death, such as fainting or unusually extreme fatigue with exercise and unexplained fainting or seizures. You should also mention it if anyone in your family experiences sudden fainting or seizures, has heart conditions including an enlarged heart, or suffered sudden death before age 50, such as from SIDS, a car crash, or drowning.
  3. Some areas offer more specialized screening for young athletes, including a test of heart rhythm (called an EKG) or even an ultrasound picture of the heart (called an echocardiogram). Such tests are considered controversial; they are not recommended by U.S. public health authorities (even though most professional and many collegiate teams require them) and they’re generally not covered by insurance. However, some groups such as the Nick of Time Foundation are collecting more and more information suggesting these screening tests are a good idea if you can get them, and I happen to agree. For more on the controversy, see here and here.
  4. Last fall, the federal government recommended that all newborns should have a simple screening test called pulse oximetry to ensure they don’t have critical heart defects, before they leave the hospital.  However, many states have been slow to adopt the testing. If you’re pregnant or have a newborn, be sure to ask your doctor about this.
Image: Heart and ecg from red ribbon via zphoto/Shutterstock.com
Add a Comment