Links for CHD Families

Here are several very important links for you to read today. Read them online, download them, or print the file, whichever you choose. If you try to download the file as a .pdf document, you won't be able to... the writers are trying to protect their work, you can read the details on the download page. But you can print the entire file, which is just as good! The only bad thing is that these links are from medical journals, written by doctors for other doctors. It's almost 100% medical terminology, and you'll probably have to have an interpreter.

Tetralogy of Fallot (ToF) in a seven year old girl

Some thoughts on Tetralogy of Fallot (ToF) surgery

The Fontan Procedure... what's down the road?

This next link refers to the Glenn Shunt, and has a very good illustration. The illustration, however, is of the Classic Glenn Shunt, which was first used in the 1950's. The modern version, the Bi-directional Glenn Shunt, was first described in 1966 and not popular until the 1970's.

Click the .pdf button to be taken to a three page file concerning both versions of the Glenn Shunt. This is important reading for anyone who has a Glenn!

This link has more layers of information than a seven layer Chocolate Cake! Above the illustration, click the heading labeled "Contents"; there you can find 40+ links to heart defects, surgical procedures, and case studies. There is lots of information here, and not just on the Glenn Shunt.

ACHA's Top 10 for Adults with CHD

Source: EmaxHealth, 11/8/08

Today, most children born with CHD have a good chance of living well into adulthood, thanks to research and innovation. However, the increasing adult CHD population poses a problem - many adult cardiologists are unfamiliar with the resulting heart anatomy and physiology that results from surgical repair. Adults with CHDs are encouraged to see specialists that are familiar and experienced with CHD. Check out Kansas City's Fox Affiliate for a recent health update and interview!

In an effort to assist cardiologists in their every day decisions for adults with CHD, the American College of Cardiology and the American Heart Association have jointly released a comprehensive set of practice guidelines. The guidelines—the first of their kind in the United States—appear in the December 2, 2008, issue of the Journal of the American College of Cardiology (JACC).

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease include:

1. Given current surgical mortalities of less than 5%, in the next decade almost 1 in 150 young adults will have some form of congenital heart disease (CHD).
2. There should be enhanced education of adult cardiovascular specialists and pediatric cardiologists in the pathophysiology and management of adult CHD (ACHD) patients.
3. Practitioners are provided with logical well-conceived care plans for patients with simple (e.g., isolated small VSD), moderate (e.g., tetralogy of Fallot) and complex (e.g., cyanotic congenital heart defects) lesions.
4. Consultation before pregnancy, including genetic counseling, so that both men and women with ACHD should have a thorough understanding of the risks of transmitting CHD to their offspring.
5. ACHD patients should carry a complete medical “passport,” listing specifics of their past and current medical history and contact information for immediate access to data.
6. A formal transition process should be used to help teenagers and young adults cross the bridge from their pediatric cardiologist to an adult cardiologist.
7. ACHD patients must be informed of their potential risk for infective endocarditis and should be provided with the AHA wallet card with instructions for prophylaxis.
8. Outreach and education programs should be organized to bring patients back into the healthcare system if they are no longer receiving appropriate care and follow-up.
9. Health care should be coordinated by regional ACHD centers of excellence.
10. Every ACHD should have a primary care physician
    

Source: Warnes et al ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease). http://circ.ahajournals.org/cgi/reprint/CIRCULATIONAHA.108.190690

Free Webcast on Complications of Adult CHD

Webcast, Patient Power®, to discuss
“Under-recognized Complications of Adult Congenital Heart Disease”
Wednesday, November 5th

6:00 pm – 6:30 pm Pacific
Oregon Health and Science University presents
“Ask the Experts” with Andrew Schorr at www.patientpower.info

Significant amounts of people are born with heart defects, many of them with congenital lesions. Because of new improvements in surgical and medical therapies, increasing numbers of these children are now patients in adulthood. Still, these patients remain vulnerable to dangers and complications of congenital heart disease. Join two Oregon Health and Science University experts, Dr. Craig Broberg and Dr. Joseph Weiss, as they unravel misconceptions and underline important factors for a disease that is often under-recognized. Both experts will help us understand long-term issues that commonly arise, the role of congenital heart disease in pregnancy, and heart rhythm disturbances. Tune in to hear a conversation with highly knowledgeable experts for a closer look into a condition many are living with.

WHO (FEATURED GUESTS):
Craig Broberg, M.D.: Pediatric Cardiologist, Oregon Health and Science University
Joseph Weiss, M.D, Ph.D.: Cardiologist, Oregon Health and Science University
Andrew Schorr: Host and leukemia survivor
WHEN: Wednesday, November 5th, 2008 from 6:00 pm – 6:30 pm Pacific

HOW TO PARTICIPATE: Listen at www.ohsuhealth.com/experts

ABOUT PATIENT POWER: Patient Power debuted in February 2005 and is a weekly show hosted by Andrew Schorr, eleven-year leukemia survivor, patient educator and patient advocate. The show features renowned medical experts on topics that include cancer, pain, diabetes, and heart specialists, as well as experts in clinical trials and top pharmacists. The show serves to bring patients together in a radio and Internet community to help navigate an often inhospitable healthcare system. Patient Power takes questions from callers and Internet listeners on topics such as how to find the right doctor, how to advocate for effectively, when to get a second opinion from a specialist, and how to evaluate one treatment option over another.

Become a CHD Advocate!

When you are a survivor, or a parent of a child that is or was affected by a CHD, you often feel helpless in a sea of insurance, legislation and lack of information. Many many of us never knew that 1 in 120 children born each day are affected with CHD before we received a diagnosis. And almost everyone affected wants to change that.

Many groups across the U.S. are working on providing support and comfort to people affected by a CHD diagnosis. But who is working with our legislators and government agencies to bring about desperately needed change? How can we create a better future for CHD survivors?

The answer? The National Congenital Heart Coalition. Formed just over a year ago, this coalition is the culmination of six major, US national groups' efforts to create a climate of change for our disease. We seek to create better avenues of awareness and advocacy, which in turn will lead to greater focus on research and technological advancements that will save lives through earlier diagnosis, better screening, advancements in quality of life and surgical breakthroughs. Consisting of the Adult Congenital Heart Association (ACHA), the Children's Heart Foundation (CHF), Children's Heart Information Network (CHIN), Mended Little Hearts (MLH), Saving Little Hearts, It's My Heart (IMH) and Little Hearts, this newly founded organization is already making strides in unifying our cause. Current projects include the creation of a unified message, creating a presence in government agencies who monitor CHD and distribute funds for research and, most recently, a unified effort in lobbying our government for a central registry of CHD statistics.

Lobby Day has been a significant effort undertaken by the ACHA over the two years. This year, the entire National Coalition (NCHC) is behind the effort, and YOU are invited to help us make a difference! You are welcome to attend the lobby day, which will be held on February 10, 2009 in Washington DC. Or, you may learn how to participate in your own local area.

Online registration is now open and available at: www.achaheart.org/getinvolved/lobbyday2009.php.

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!

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.

Long-term Care of CHDs

As a parent of a child with Transposition of the Greater Arteries (TGA), I will never forget the moment when the surgeon sat down with me after her open-heart surgery and told me "She's fixed." When I asked what I could expect from then on out he told me "She'll be able to live a normal, active life" and "she'd only need a check-up once a year or so." What he didn't tell me is that she was NOT fixed. She will always need to be watched closely. It is likely that she will face complications down the road.

Sure, it's every pediatric cardiac surgeon's dream to tell a parent that a child is "fixed." It doesn't happen often. Some of the more severe defects require many surgeries, none of which are a "fix," but are aimed at extending the lifespan until a fix or alternative treatment can be found. But is it really fair for a surgeon to sugar-coat the truth? No, it isn't.

That's why I was upset when I found out that my daughter's defect is considered severe, requiring life-long care. I had entered into a world where TGA seemed like a minor defect. The truth was like a slap in the face that woke me up from my complacency.

The truth about Congenital Heart Defects is that many DO require life-long care and follow up - not with an adult cardiologist (that is often not specialized in CHD-care), but with a specialist in Adult Congenital Heart Defects. The Adult Congenital Heart Association is working to spread the message, as are many CHD specialists across the world.

For an example, check out today's (17 June) news article from KSPR News in Springfield, Missouri:

Heart Defects need medical attention through adulthood
By KSPR News (visit the link for video footage)

Fifty years ago babies born with congenital heart defects weren't expected to live into adulthood. But thanks to advances in medicine, most babies with heart defects now live long, full lives.

"What it's called is tetralogy of fallot. It's one of the most common occurring complex defects."

Kyra's heart had four abnormalities. The pulmonic valve was narrowed, and there was a hole in the septum, or wall between the right and left ventricles. Also, a main artery was in the wrong place. These issues caused her right ventricle to get thick because of all the extra pumping it had to do. At age 4, Kyra had surgery. She was considered cured.

"Then I started developing symptoms. Shortness of breath, electrical conduction problems, arrhythmias."

It turns out she needed another valve replacement. The repaired valve started to leak. Dr. Keith Oken says Kyra was a victim of a common misconception.

"Which is that she was cured with the surgery she had. That is rarely the case."

Kyra needs continuous, life-time care. But there are not many cardiologists trained to manage cases like hers. That's because until recently most people with congenital heart defects did not survive into adulthood. So to make sure Kyra stays healthy, she sees her regular doctor and experts at mayo clinic. Plus she's doing whatever she can to keep her heart strong...

Doctor Oken says people with adult congenital heart defects need to continue seeing their doctors... And to see a heart defect specialist.

CHD and Neurodevelopmental Risks

Risk of attention and behavior problems for kids with CHD
Children's Hospital of Philadelphia
Public Release, 7 April 2008

Schoolchildren who required surgery as infants for congenital heart disease (CHD) run a significant risk of having problems with inattention and hyperactivity, and often require remedial services in school. “These children are at risk for academic and behavior problems, and our findings reinforce how important it is to provide them with ongoing follow-up and neurodevelopmental screening,” said study leader Amanda J. Shillingford, M.D., a pediatric cardiologist at The Children’s Hospital of Philadelphia.

The researchers, who reported their findings in the April issue of Pediatrics, studied a group of 109 children, aged five to 10, who had undergone cardiac surgery for complex congenital heart disease at Children’s Hospital when they were newborns. Of that group, 53 children--nearly half of them--were receiving remedial services at school, and 15 percent were in special education classrooms.

Based on questionnaire responses from their parents and teachers, although the majority of the children with CHD scored in the normal range, the rates of high-risk scores for inattention and hyperactivity were three to four times greater than those found in the general population.

Previous studies at The Children’s Hospital of Philadelphia and other centers found that school-aged children with complex CHD tended to have normal cognitive abilities but were at risk for problems in visual and motor skills, as well as impairments in speech, language and executive functioning (executive functioning refers to capacities for attention, planning, decision-making and problem-solving).

“As survival rates have improved,” said Shillingford, “the important longer-term issue is quality of life for patients and their families as they reach school age and beyond. We hope our findings will help raise awareness among parents, teachers and physicians about the children’s risk of neurodevelopmental problems.” Shillingford added that a next step for researchers is to conduct larger, multicenter studies, with more formalized diagnostic tools, and to develop formalized follow-up protocols for these children. Such follow-up programs are currently being designed at Children’s Hospital.