Children With Broken Hearts

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/
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  1. by Kaitlyn

    On May 3, 2012 at 2:53 pm

    Thanks for this article. My son was diagnosed with Wolff Parkinson White syndrome when he was 15 days old. When we finally got him to the pediatric ER, his heart rate was 290 bpm! We are so thankful for his pediatric cardiologist. We just wish more information was published on WPW and more people talked about dealing with the unknown and heart conditions that may exist in children without you even knowing. We’ve written a few articles about our experiences at It’s Learning Time

  2. by Helene

    On May 5, 2012 at 8:04 pm

    Thank you for sharing this. It’s so very important to spread awareness about these things.

  3. by Around the Web… |

    On May 9, 2012 at 12:25 pm

    [...] The hidden problem of heart defects in children – [...]

  4. by Sarah

    On October 30, 2012 at 9:47 am

    Thank you so much for preparing CHD awareness! Unfortunately most people do not know that 1 in 100 babies will be born with some type of CHD. Our son has hypoplastic left heart syndrome and while we were lucky enough to find out prenatally most parents don’t know something is wrong until they get home and their baby starts breathing faster, turns blue, etc. a simple pulse ox test before being discharged from the hospital can save many of these babies.

  5. by Dawn

    On October 30, 2012 at 9:58 am

    One of the biggest things that I have desired to see happen is the spreading of CHD awareness. This article helps this cause, because sadly, there are still many many people who have never heard of this number one birth defect or how to screen for many heart defects. I live in Colorado and here pulse oximetry screening is controversial. Many of us “heart parents” are just as passionate about mandatory screening as we are about spreading awareness about these defects. I didn’t learn about CHDs until 5 1/2 years ago when my second-born was diagnosed at 2 weeks old. Thank you Parents for posting this!

  6. by Wendy

    On October 30, 2012 at 11:30 am

    Thanks for publishing this article. Too often parents are not aware that congenital heart defects and other electrical issues of the heart are a possibility for their child. I have one son with a heart defect that has required 5 heart surgeries so far, that wasn’t diagnosed until he was 5 years old and another that was diagnosed with long qt when he was 12. One in a hundred children are born with a CHD and more children die of heart disease than all childhood cancers combined. It is a real problem but people think it is so rare and will not affect them. We need more awareness and funding to find answers. Thanks for your part in raising this awareness.

  7. by Erin Ayscue

    On October 30, 2012 at 6:24 pm

    So VERY happy to see this article! Our daughter was born with heterotaxy syndrome, including 14 congenital heart defects. I had no clue until her birth that CHD’s are the #1 birth defect world wide. Too many babies are dying from undiagnosed CHD’s, and many were unnecessary, preventable deaths. Every single person of childbearing age, pregnant mothers and mothers of newborns should be aware of CHD’s, the signs and sympotms, and that a pulse oxemetry test at birth can prevent horrible heartaches and tragedies. Our daughter wasn’t supposed to live but a few hours, but she is now five. She has had three heart surgeries, has a pacemaker and many other issues, but she is ALIVE.

  8. by Brendon

    On October 31, 2012 at 4:45 pm

    This is very good information, but I have a question I’m hoping someone can help me with. I’m totally serious & not trying to be smart. Maybe I read it wrong, but how is death of a family member in a car crash or drowning related to a child’s heart problem?

  9. by Christen

    On November 1, 2012 at 3:30 am

    In 2007 my then boyfriends mother died unexpectly at the age of 40 from hypertrophic cardiomyopathy ( HCM) . At that time it was suggested that the immediate family be tested. My know husband got diagnosed with HCM in 2008. Later that year my mom a nurse was talking with some colleges about his diagnosis and he was suggested that he see MD Berry Marron at the Minnesota Heart institute in Minneapolis Minnesota. The specialist for HCM. This Cardiologist has specialized in this conditions for more then 30 years. Written hundreds of research articles and a patient education book on the disease. What people may no know is that although most cardiologist can detect HCM they see very few cases and are unsure of how to treat the disease. Within months of seeing Marron my husband was taken to surgery and a defibulator was placed in his chest to hopefully prevent the sudden death that happened to his mother from ventricular tachycardia. If you know of someone with HCM I would suggest that they make a trip to Minnesota to see this doctor it may end up saving there life. Thank god the defibulator has not been needed but if that day comes he has a chance of making it out alive. Now 3 years after the surgery we have a healthy baby girl and looking into gene testing for HCM to see if we can not have the gene located and our healthy baby cleared from the fear of someday being diagnosed with HCM.

  10. by Christen

    On November 1, 2012 at 3:33 am

    To the person who is asking why crashes and drowings have to do with Cardiac events. Offen a cardiac event happens and cause on of these event to fallow. In the case of my husbands mother she was driving a car when she had her cardic event and there for crashed it. if the case of death is listed wrong members of the family may be at risk and not even know it.

  11. by Teri

    On November 1, 2012 at 10:48 pm

    Our daughter was born with a ‘broken heart,’ more specifically Transposition of the Great Arteries and multiple ventricular septal defects. (TGA/VSDs) She was 1 week old when her defects were found, only because we had gone in for a billirubin check. She had the Arterial Switch (open heart surgery) at 22 days of life.

    Our best advice to anyone preparing to have a child, just as the article says, ask for a pulse-oximetry test! Make sure they do it just before you leave the hospital–in addition to shortly after birth. 24 hours can make a signifcant difference in their pulse-ox numbers. Our daughter’s blood was able to oxygenate because of the VSDs, but we were told she would have died before her 2wk check up. We wish we’d know to ask for the test–it’s simple, painless, quick and free!! She is our “1 in 100″…but to us, she is one in a million! We are blessed!!