CHD Death Incidence in Infancy

Source: Patient Health Information, 13 October 2008

MedWire News: A significant number of babies are dying soon after birth because of undiagnosed congenital heart disease, a US study suggests.

To examine the extent of the problem, researchers from the University of California used the California death registry for the years 1989−2004 to assess data on 898 infants who died of congenital heart disease between birth and 1 year of age.

They found that, in total, 152 of the babies had a missed diagnosis of congenital heart disease. The average age at death was 14 days and more than half of the babies died at home or in the emergency department, indicating that they had been discharged from the postnatal ward. The team concluded that current screening methods are inadequate.

That's roughly 1 in 8 deaths with a condition previously undiagnosed that died at about 14 days. It is not clear that these children could have been saved if they had been diagnosed on time. Only time, and the creation of an adequate registry for CHD, will provide these answers.

This is one front that all CHD organizations, large and small, should be united on - creating an adequate survey of patients, births and deaths related to CHD, so that we can truly begin to understand this series of anomolies.

The Moral Obligation to Give Back

If you haven't yet subscribed to Adventures of a Funky Heart, (written by an adult CHD survivor and packed with humor and insight), I highly encourage you to peruse the interview he recently conducted with Amy Verstappen, President of the Adult Congenital Heart Association (ACHA) and a survivor of Congenitally Corrected Transposition of the Great Arteries. In her interview with Steve, Amy discusses her "moral obligation" to give back to the CHD community after her personal experiences as an adult survivor. Her personal story is an interesting one, but the action she has taken is nothing short of inspirational.

In the past year, the ACHA has recently joined with several other Congenital Heart Defect organizations to form the National Congenital Heart Coalition. Amy is actively involved in creating new legislation to bring much needed research and attention to CHD issues.

“I am 100% positive that in the long run we will have an integrated, high quality care system for all survivors,” Amy says in the interview. “Our really long term goal is to have a very unified system with research, a registry, a way to quantify outcomes, all the centers talking to each other, sharing information and building knowledge together...”.

Ms. Verstappen is determined to educate parents on the long-term needs of CHD patients. She tells Steve that she “...presented a pilot version of [a new brochure designed by ACHA specifically for parents] to a group of Heart Moms - all leaders of some of the advocacy groups for children - three of them told me that no one had ever told them that their child was going to need this level of care.” Amy goes on to describe some of the questions that parents of children with a heart defect should be asking.

I can tell you, as one of those moms who didn't know or understand the long term implications (I have a child with Transposition of the Greater Arteries who I was told was "fixed!"), that the information provided is valuable and too important not to take a moment to read.

I highly encourage you to take a minute and check out the latest entry at Adventures of a Funky Heart! Not only is it a great personal story of one of the movers and shakers of the CHD community, but it is also a great source of information for parents, teens and adults alike!

Online Discussion on Adult CHD to be held October 2

Next Thursday, October 2, 2008 at 12 noon (EST) Cleveland Clinic will host an online health chat on Adult Congenital Heart Disease.

During this chat, Dr. Richard Krasuski, Director of Adult Congenital Heart Disease Services and a staff cardiologist in the Section of Clinical Cardiology, Department of Cardiovascular Medicine at Cleveland Clinic Heart and Vascular Institute, explores the treatment options available for adults living with a congenital heart disease. More information on this chat can be found at http://www.clevelandclinic.org/health/ChatReg/Krasuski.html.

Dr. Krasuski's specialty interests include adult congenital heart disease including atrial septal defects, patent foramen ovale, ventricular septal defects, coarctation of the aorta, Ebstein anomaly, Eisenmenger syndrome, transposition of the great arteries, Tetralogy of Fallot and patent ductus arteriosus. He also specializes in pulmonary hypertension, mitral valve disease, cardiac catheterization, transesophageal echocardiography and mitral, aortic and pulmonic valvuloplasty.

Too Close To Home...

A recent article in The Sun (13 Sept) asserts that a teenage girl with a congenital heart defect died after telling her gym teacher she did not feel well. She was warming up for hockey practice when she asked to stop because of chest pain but, according to classmates, the gym teacher told her to keep going. The classmates then watched her collapse on the pitch.As a heart parent, I can tell you that this is one of my biggest nightmares. I've been unable to let go of the bond with my daughter into another's care (other than family members and the occassional night out while the neighbors come over). Granted, she's only two at this point, but I worry about the day when she will enter public or private school, out from beneath my watchful eye. I've heard so many stories about children with special directives and orders who are overlooked or ignored by their teachers, counselors, coaches, etc. How will I protect my daughter from such a tragedy, yet encourage her toward independence?

Of course, no one can fully protect their child. I understand this. But one can take steps to help ensure a tragic accident such as this one does not occur. The parents of the girl who passed away at hockey practice had made the school aware of Sasha's condition, so they assumed that this information would be passed on to her teachers. Apparently, it was not.

As parents, it is our responsibility to teach our children how to live with their heart condition, to know their bodies and be able to judge accordingly when "enough is enough." The father of the deceased child said, “Sasha had lived with her heart condition since she was born and she knew her own body. Yet even though the school knew she had this problem, they still didn’t listen to her when she said she was unwell.”

Clearly there is something to be learned from this tragic incident. I know I personally take the following from it:

1. Teach your child about their heart condition. Help them to understand their body.
2. Make sure your child's caregivers are aware of his or her condition, and the potential stresses that could lead to a serious medical crisis.
3. Teach your child that it's OK to take breaks when you need to. Even if a coach, friend or other person is pushing you to go farther, your child is the best judge of his/her body. Don't take unnecessary risks.
4. Talk to gym teachers, coaches and other sport instructors that your child will be working with. Make sure they understand the child's condition and any special considerations and precautions that should be taken.
   

What do you do to protect your child? How have you taught him to read his body's signs? How do you help her to engage in a healthy, active lifestyle without providing undue stress to her already fragile heart?

Real CHD Survivors on Reality TV!

GROWN UP “HEART KID” FEATURED ON SEASON FINALE OF LIFETIME TELEVISION’S HIT SERIES “HOW TO LOOK GOOD NAKED"

PHILADELPHIA – SEPTEMBER 04, 2008 – The Adult Congenital Heart Association (ACHA) announced today that congenital heart defect (CHD) survivor, Heather Magee, will be featured on the season finale of Lifetime Television’s hit series, “How To Look Good Naked,” airing September 9 at 10:00 PM EST.

Magee, a survivor of two childhood surgeries to correct a congenital heart defect that left her with only one working ventricle, the most recent of which was performed in October 2007, confronts her body image on the show in the wake of multiple scars, both physical from the incisions, and the mental impact of coping with a congenital heart defect as an adult.

“For almost twenty years I lived under a false sense of security that my heart condition was a childhood problem that was fixed long ago,” said Magee. “When I discovered four years ago that CHD is a lifelong struggle that requires ongoing care, it threw me for a tailspin. Not only did I suffer physically from my condition and the resulting corrective surgery, but mentally as well. I participated in ‘How To Look Good Naked’ both to raise my self esteem after a traumatic time in my life, and also to raise awareness among the millions of adults living with CHD that they must seek treatment for their condition if they aren’t already doing so.”

Approximately 1.8 million adults in the United States are living with congenital heart defects. Most, like Magee, underwent open-heart surgery as an infant or child, and drop out of special congenital heart care, as they believe themselves to be “fixed.” However, recent evidence shows that heart procedures performed on young children may weaken over time, leading to serious health complications as early as young adulthood.

Magee’s appearance on the season finale of “How To Look Good Naked” will do more than just increase awareness of congenital heart defects among the viewing audience, it will help raise the funds necessary to continue the ACHA’s mission. For the first time in the series’ history, the episode also served as a fundraiser. The show featured Magee and other adults living with CHD in a charity fashion show that raised $10,000, which will be divided between the ACHA and the Ahmanson/UCLA Adult Congenital Heart Disease Center (Ahmanson/UCLA ACHD Center), where Magee was treated for her condition.

More about the show can be found at Lifetime's website.

For More Information:

Anna Espe, Development Associate
Adult Congenital Heart Association
215-849-1260
aespe@achaheart.org

The Tallest Kid in the Room

There's a great new blog out there that I'd love to point our readers to... Adventures of a Funky Heart. The blog is written by Steve, a 41-year old survivor of Tricuspid Atresia. He's allowed us to reprint a portion of a recent entry here. Enjoy, and be sure to check out Adventures of a Funky Heart!

July 28, 2008 by Steve

They always have Highlights for Children. It’s a common staple of any Pediatrician’s office, but it never seemed that they made the magazine available for home delivery. I always wanted a subscription to Highlights, because I enjoyed all the puzzle pages. But that was then. Times have changed, and now I’d rather hae a subscription to a good baseball magazine. Most children aren’t that interested in baseball, so I need to bring my own.

I walk into the doctor’s office and I can feel the eyes boring into my back. I can’t see them, but I know that everyone is looking at me. When someone my age comes into a pediatrician’s office without a child, everyone stares. What’s he doing here? If they are wondering now, just wait until the nurse checks me in and tells me to have a seat.

Usually all of the adult chairs are occupied. It’s impolite to stand in the waiting room – there’s no rule against it, but I’ve never seen it done unless every seat is taken – so I sit in one of the children’s chairs. That’s an adventure unto itself. You sit down as you normally do, but you just keep going down until your bottom hits the chair with a bone jarring THUMP and your knees are level with your eyes. Now I look stupid, so I stretch out my legs. Not only am I blocking the walkway, but now I really look stupid, so I pull my legs back in. Hopefully this won’t be as bad as the time that I had pneumonia: my doctor admitted me to the hospital, and after a 4 hour wait in the ER I learned the only available bed was on the Children’s wing. It was a loooooooooong way from my rear end to the child sized toilet, and I was too far down to stand up! I literally had to roll off the seat and then get to my feet. But there was no room in the inn, you take what you can get, and beggars can’t be choosers.

Until I was 30 years old I saw my local pediatrician for non-emergencies. It works the same way for kids with heart problems. “Adult” Cardiologists can’t deal with us – they are trained to deal with heart attacks, clogged arteries, and all the problems that your heart develops as you age. They usually don’t have experience dealing with Congenital Heart Defects (CHD). A friend of mine – also a Cardiologist – once said that if you chose to specialize in Adult Cardiology, you received about two hours of training in CHDs. Basically just enough to know that they exist. So no matter our age, Cardiac Kids are still patients of a children’s doctor.
But now there’s a new specialty, known as an Adult Congenital Heart Defect doctor. Adult Congenital Defect care is not taught at any medical school (yet); many of the ACHD docs are really Pediatric Cardiologists. Since their patients stay with them practically all their life, these “children’s doctors” found themselves dealing with questions about work, pregnancy, dating, insurance… questions that Adult Cardiologists usually have to answer. And so the field of ACHD Cardiology was born.

You won’t find one on every corner, but ACHD care can be found. Usually, you’ll need to go to a major medical center. I live in rural South Carolina; the ACHD centers in the South include Duke University, Emory University, and the University of Alabama at Birmingham (UAB). Other major centers include The Mayo Clinic, Stanford, the Philadelphia Adult Congenital Heart Center and Children’s Hospital of Boston. There are other clinics, of course, but those are the ones that spring immediately to mind. And now, the waiting rooms have people who are more in my own age range.

I miss the visits to the Pediatric Cardiologist, though. While I’m sitting there trying to get comfortable, absorbing all the stares, the kids don’t pay a bit of attention to me. Kids are kids, they are usually congregated in the play area, getting along famously and having a great time. For once, they aren’t being left behind on the playground – everyone is moving at about the same pace.

The parents are the ones sitting there with the worried look on their face, with good reason. I always hope that one of the parents will speak to me, perhaps ask if my child is sick. If they do, I’ll tell them that no, I’m the one with the heart defect. My parents were in the same boat you were. I’m 41 now, and still going strong. And hopefully someone will come to believe that with good medical care and a little good fortune, their child will do well.

Research Yeilds Answers on Aortic Arch Defect Causes

By The Pittsburgh Tribune-Review
Tuesday, July 8, 2008

Research led by University of Pittsburgh scientists is the first to describe how two common congenital circulatory problems form, the university announced today.

The team found that a gene called unc45a plays a critical and previously unknown role in the formation of aortic arch vessels. The vessels contribute to several of the body's major arteries and often develop improperly, causing a wide range of vascular defects.

The team also found that arteriovenous malformations, or AVMs, happen when an artery fuses with a vein, diverting blood flow, and result from genetic and physiological factors. Previously doctors had believed its origins were solely genetic.

The research is published in the journal Developmental Biology. Scientists at Georgetown University Medical Center and the National Institutes of Health also participated.