Meet Andrew!

About a month ago, I was contacted by a young man with HLHS with a great deal of wisdom and a great story to tell.  Meet Andrew, HLHS survivor, age 22. He is a college student studying anthropology, enjoys traveling, competitive road cycling, and has a goal to advance patient advocacy in healthcare.  Andrew was kind enough to answer what I think are common questions asked by parents of children with HLHS. 

Q&A:

How old were you when your parents first explained to you that you had a heart defect? Do you remember what they said?

I don’t remember a time when I didn’t realize that I had something which made me a bit different. My parents must have been pretty open with me as soon as they could. I don’t think I really understood it until maybe around 1st or 2nd grade?

That’s when I started to realize/learn that it was my heart which made me different from other kids I knew. After long, I was separated during rough physical activity, which clued me in some more.

Do you remember if when you were in elementary school or high school you had a hard time keeping up with your peers? How did you handle this?

I kind of ran wild with this in high school. Years of being removed from physical activity let me explore my inner audio-visual nerd. I worked on the high school TV shows, and even ended up getting paid to film, edit, and burn DVDs for school functions and productions.

Since I never was able to keep up with the other kids physically, I did something which let me still interact with them using what I could do. If my friends were doing some activity that I couldn’t, I would film or photograph it. The big ones were team sports, and some really crazy mountain-biking (with lots of hard falls). It was a great way for me to get included in the wacky group-dynamics of high school.

Did you feel different from other kids because you have HLHS?

I definitely did. I never really felt negatively about it though. I was almost proud of it. Perhaps it was the super-liberal atmosphere of where I grew up, where diversity and understanding were championed just about everywhere.

Have you ever been self-conscious about your chest scars?

My scars always bugged me until I was about 16-ish. I'm not sure if this is typical of HLHS patients, but something happened to my sternum after my surgeries and it came to a bit of a point, which splayed my pectoral muscles out to the sides a small amount, and my rib-cage is a bit more visible than on a 'normal' skinny person. I remember one summer when I was still pretty young a Lands End catalog came and they had these lycra swimsuits for kids, styled after wetsuits, which zipped up all the way to my neck, covering your chest. My Mom ordered me one and I lived in that thing whenever I was doing any type of aquatic activity. (Sailing summer camp or an evening on the boat). I felt pretty fancy in it too, how many kids get to wear a wetsuit? Ha. 

Before I had the wetsuit (and after I outgrew it) I would always just keep a shirt on in the pool, which wasn't all bad... it saved me a lot of sunburns. I eventually started not to care if people saw my scars and went without. I always had a story handy if someone was going to hassle me about them though. I used it once, and it worked pretty good. It was a school field-trip to a local pool towards the end of the year- and some of the 'cool' kids were picking on my scars and I told them that I got shot and that is where they took the bullets out of my body. I don't recommend it, but it worked to get them to leave it alone!

I think it takes everyone, especially young kids with any kind of physical abnormality a while to accept, and believe the idea that it doesn't really matter what other people think about their body. 

How is it to be a young adult with HLHS?

I’d say it’s pretty much the same as being a ‘normal’ young adult. I just have to take medicine, go to my cardiology appointments, and be a bit careful with how I treat my body.

Do you ride on roller coasters?

That was one of the things I was always told I couldn’t do. My cardiologist said in no uncertain terms that I shouldn’t ride roller coasters. I’m perfectly fine with that, I like for my lunch to stay in my stomach anyways. This also included never being in any other no/low/high-gravity situations, but I’m pretty sure I have that covered as long as I stay here on earth...

Do you play sports?

I love cycling. Strangely enough, I didn’t learn to ride a bike until I was around ~12 or so, I suppose that’s a bit later than ‘normal’ but it worked for me. The summer I turned 13 I remember I got a new bike and I loved it. I rode that thing everywhere, all over town. Our community had some really extensive bike paths, which you could ride for about as long as you wanted. I think that summer I hit around 150 miles. I was super proud of that.

Cycling is still my favorite sport, I’m able to out-compete other people my own age, which certainly doesn’t hurt the ego. February of ’10 I got a new bike (bike sales in the winter!) and I think by September I had reached close to 500 miles in total.

Shortly after hitting my 500 mile mark, I was in a really serious accident which put me on a temporary cycling hiatus. I ended up breaking three ribs, and shattering my left kidney to bits. It landed me in the ICU for a week, and bed rest for about a month after. (I had a helmet on, or else I’d probably be dead)

After this, I thought I’d be done with cycling for good and sold all my bikes and anything bike related. It’s more than a year later and I’m still dealing with some pesky stiffness and soreness from that accident, but this summer I did do a little bit of riding. Nothing as serious or as fast as before, just some moderate exercise. I decided that I couldn’t ditch my favorite form of exercise. I think next spring I’ll be back in the saddle and I really want to break my 500 mile mark.

I actually read an article on pubmed awhile ago, (which I cannot find now) about why cycling is a great activity for people with heart issues.

Do you take any heart medications? Do you feel any side effects because of your medications?

I take 10mg of Lisinopril and an 81mg aspirin daily. I don’t seem to notice many side effects unless I miss a dose, if I do miss a day I feel a slight tightness in my chest.

What has your doctor said to you about your heart in the future? Will you need any other surgeries?

My doctors try not to predict the future, which I appreciate. Living with something like HLHS requires you to kind of go with the flow. If a doctor were to tell me “you’ll be fine, you’re fixed!” that would be great, but then if he had to tell me “so...about that...I was wrong” a year later, it would really be a bummer. As it stands now they’ve left open both options; I might need more work, I might not. They’re not sure. They’ve even recently brought up the idea of looking into a full transplant.

I check in twice a year with my cardiologist (or as needed for things which come up) and we go from there. I’ve just recently ‘graduated’ from a pediatric cardiologist to an adult cardiologist. Finally, no more waiting rooms full of coloring books!

What has your doctor said to you about drugs, smoking and alcohol?

My doctors (GP & Cardiologist) both said the same thing. You’re an adult, you can do what you want. With that being said, they both advised me to not partake. I’ve taken their advice. I’m pretty strict about it, I think it’s a personal choice that each person has to make.

I figured, I’ve been lucky enough to be quite healthy for an HLHS patient, I probably shouldn’t mess with it.

*This includes caffeine, which I do not consume in any amount- including what’s naturally found in chocolate or certain teas.

What have you been told about birth control and having children in the future?

I’m not too sure how keen I am on having a child of my own. That might just be the 20-something-year-old male in me speaking though. I have thought about it, and if at some point in my life I’m struck with the desire to be a parent, I’d look into adoption. I do still have some reservations, just simply due to things like the fact that the lifespan of adult HLHS patients is still somewhat unknown.

What are you plans for work in the future?  Has your diagnosis ever been a consideration when thinking about a job? 

After I finish up my undergrad degree, I want to go to graduate school, and get a masters degree in public health. With that degree I would like to work for some type of organization (or hospital) dealing with patient advocacy. Being a patient my entire life has had a profound impact on my desire to work on this field. I’ve seen the ins and outs of the system as a patient. There are many areas of our system which could be changed to make it easier on patients. Since we have the ability to make most of these changes, it would be silly not to.

What is the most important thing you would like to share with kids about living with HLHS?

You’re not all that different than a ‘normal’ person, don’t let people tell you otherwise.

Thank you Andrew for sharing with us! 

Sharing is Caring...

I remember the exact second when I first heard the words - your baby has a heart defect. There is no doubt in my mind that I had the deer in the headlights look on my face. In one instant, everything had changed, and as I reflect on that moment in time, I am certain that right then I began the grieving process for my unborn child.  Those feelings lasted throughout the rest of my pregnancy, and were pitted against my feelings to fight for my baby's survival.

I see that same look on parents’ faces all too often in my line of work. My professional position centers me in high risk OB where we care for the pregnant mothers of future heart babies. Being a parent of a child with HLHS, and working where I do, does not automatically qualify me as a person to consult with. I very rarely share my personal story with the patients I meet for research studies. Like most do when working in a clinical setting, I try to maintain a separation between work and home, but every now and then I meet parents that are in such duress I am compelled to offer a glimmer of hope, and I do so very carefully. If you are a parent of a child with HLHS, and your child has been through the staged surgeries, then you understand why it is important to be guarded with your experiences. They are often too much for a future HLHS parent to bear, and outcomes are very different for each child. But our experiences have put us into a very unique position, and as a member of several heart groups where I often meet newly diagnosed parents, I hear a lot of questions from parents, many of the same questions I once had myself. So I share what I've learned.

What surprises me today is hearing from parents their story of diagnosis, and how pregnancy termination is offered as an option, as HLHS is considered a lethal anomaly. Meaning, pregnancy termination can be administered in the hospital setting as a medical termination based on lethal anomaly. Please note that I am in no way judging anyone who chooses this option - my focus is on the terminology - lethal anomaly. A diagnosis of HLHS is no longer a bleak prognosis so it is difficult for me to acknowledge that it is a lethal anomaly. It is if no course of action is taken, but through staged palliation or heart transplant, the survival rate is very good at 90-95% and 80% respectively.  

I often have heart parents in the early stages of palliation ask me questions about my older HLHS child.  This is said of many experienced heart parents. So how did we become the “experts?” By default I suppose. Who better to talk to about everyday life with a heart baby than a parent who has been through it?  And I like the idea of sharing. I like that parents feel the comfort and reassurance that there is hope. Sometimes, hope it all we have. Consider being a mentor to new heart parents through a parent matching program. Some of my favorites are: It's My Heart, Kids with Heart, Little Hearts, Mended Little Hearts, Saving Little Hearts, and Sophie's Heart, but these are just a very few as there are hundreds of parent-matching programs available worldwide, and there may even be one through your child’s very own heart hospital.

Sharing is caring... 

Making Your Way Through the Disability Maze...

On July 14, 2011 the Social Security Administration (SSA) added Hypoplastic Left Heart Syndrome to Compassionate Allowances (CAL) which is a list of medical conditions that meet disability standards allowing Social Security benefits to be provided quickly to those who qualify. The benefits program is called Supplemental Security Income (SSI), a federal income supplement program designed to help aged, blind, and disabled people, who have little or no income; and provides cash to meet basic needs for food, clothing, and shelter.

Applying for this program is not for the light-hearted. It is a maze of paperwork, and it may take months or even years to obtain approval. In many cases, initial claims are denied and the process must be continued through the appeals process. Having just enough insurance and just enough income, we were not eligible to receive the benefits of this program, however, we were encouraged to apply, and I encourage you to at least learn about the programs available. Circumstances change and it’s good to at least know what the process entails.

On the SSA Website you will find The Child Disability Starter Kit which includes a Factsheet titled, “What You Should Know Before You Apply for SSI Disability Benefits for Your Child,” which answers questions about applying for SSI child disability benefits, a Checklist titled "Child Disability Interview,” which lists the documents that you will need for the disability interview (in person or online), and a Worksheet titled, “Medical and School Worksheet – Child,” which lists the information you will need about your child's medical condition to help you to prepare for the disability interview (in person or online). 

Once approved for the SSI benefits program, your child will receive Medicaid (Title 19) insurance and cash benefits. Your child may have to participate in a Continuing Disability Review (CDR) every few years to continue receiving benefits. During a review, the SSA will review your income, resources, living arrangements and your child's medical condition. This process is called a Redetermination, and it may be scheduled or unscheduled. 

If your child was denied SSI, don't give up! You have the right to appeal the decision and the right to obtain assistance with refiling your claim. 

Just recently I learned about a state program called CHIP, the Children’s Health Insurance Program, which provides health insurance to children whose parents have incomes too high to qualify for Medicaid, but too low to afford private health insurance. The CHIP program provides insurance coverage for prescription drugs, vision, hearing and mental health services and is available nationwide.

Undoubtedly, the Disability process is a maze of paperwork, but there are many resources available to help you along the process. The Department of Justice provides information on your child's rights on it's Americans with Disabilities Act website, the Department of Labor addresses your rights as a working parent through COBRA and the Newborn Act, and the Department of Health and Human Services provides information for families regarding health, financial assistance and health insurance. 

No matter what the circumstances are, caring for a child with a heart defect is not an easy task. In addition to the emotional stress that is present, the financial pressures can be overwhelming. There is the prospect of lifetime care, the likelihood of increased medical bills, and often special equipment to properly care for your child. Fortunately, there are options for parents caring for a child with a heart defect. If you do not qualify for federal or state assistance programs, do know that there are resources available to parents for prescription assistance, financial assistance and health insurance assistance. Talk with the social services department at your child's hospital, your child's primary care physician, and your child's cardiologist.  The Congenital Heart Information Network Healthcare/Insurance Resource page is also an excellent source for parents and provides many links to resources.  

BACK-TO-SCHOOL CHECKLIST!

School Shopping List: Markers, Glue Sticks, Pencils, Hand Sanitizer, Pulse Oximeter, Defibrillator (AED), Individual Education Plan (IEP)….. Does your heart kid have everything on his/her list?  

Some school necessities are easier to come by than others.  Many schools currently have a Pulse Oximeter in the nurse’s office and several states require AEDs in their schools, although the requirements vary by state. Some states require teachers or school coaches to be trained in AEDs use and others offer grant money to encourage schools to purchase AEDs. The state of Oregon recently passed legislation that mandates AEDs in schools by 2015. New York, Illinois, and Pennsylvania are now mandating the placement of AEDs throughout their schools and AED legislation is pending in many other states. House Bill 1919/Senate Bill 2095 would require AEDs in middle schools, high schools, colleges and universities beginning with the 2012 school year and in elementary schools by the 2014-2015 school year. Click here to help your school launch an AED program.

Another important item on the back-to-school checklist is knowing your child’s educational rights. According to the Department of Education, the Individuals with Disabilities Education Act (IDEA) requires all public schools to develop an Individual Education Plan (IEP), also known as the Section 504 plan, for every student with a disability who is found to meet the federal and state requirements for special education. The IEP must be designed to provide the child with a Free Appropriate Public Education (FAPE). The IEP refers both to the educational program to be provided to a child with a disability and to the written document that describes that educational program. At the end of twelfth grade, students with disabilities will receive an IEP diploma if they have successfully met the IEP goals. If they have met the requirements for the high school diploma, then it may be given in place of the IEP diploma.

The IDEA requires that an IEP must be written according to the needs of each student who meets eligibility guidelines under the IDEA and state regulations, and it must include the following:

• The child's present levels of academic and functional performance
• Measurable annual goals, including academic and functional goals
• How the child's progress toward meeting the annual goals are to be measured and reported to the parents
• Special education services, related services, and supplementary aids to be provided to the child
• Schedule of services to be provided, including when the services are to begin, the frequency, duration and location for the provision of services
• Program modifications or supports provided to school personnel on behalf of the child
• Least Restrictive Environment data which includes calculations of the amount of time student will spend in regular education settings verses time spent in special education settings each day
• Explanation of any time the child will not participate along with nondisabled children
• Accommodations to be provided during state and district assessments that are necessary to the measuring child's academic and functional performance
• The student should attend when appropriate. If the student is over 14 they should be invited to be part of the IEP team.
• Additionally, when the student is 16 years old, a statement of post-secondary goals and a plan for providing what the student needs to make a successful transition is required. This transition plan can be created at an earlier age if desired, but must be in place by the age of 16.

IEPs also include other information such as a healthcare plan or a behavior plan for some students.  For children with a CHD, an Individual Healthcare Plan (IHP) is developed by the school nurse with written input and approval of the primary care physician and other healthcare providers. The IHP identifies your child’s medical needs at school and how those needs will be met. The team considers the nature and complexity of the child’s condition to ensure their health and safety.  Factors include support needed during the entire school day, including the classroom, physical education, the school bus, behavior needs, field trips, and extracurricular activities. 

Speak with your child’s health care providers about his/her specialized needs in school and request that they document your child’s needs in writing to share with the school. Request a meeting with your child’s school nurse to discuss the development and implementation of an Individualized Health Plan for your child.

Once your child’s IEP/IHP is developed it is important to remember the following:

• Ensure that training is provided
• Ensure the IHP is being implemented
• Contact appropriate staff periodically to ensure plan is working
• Check with your child frequently
• Keep your child’s health care providers informed
• Inform your school of any changes
• Update the IHP at least annually

Best Wishes for a happy and successful new school year!

A Disclaimer

For those of you who know me personally, you know why I began this blog. For those who do not, I'd like to tell you "why."

First off, it's funny to me to say that I blog - no offense to my fellow bloggers out there - I subscribe to many beautifully written blogs and you don't write fluff!  But I have a science degree, I've worked in the healthcare industry since I was 18 years old, and I've been in clinical research for several years, therefore when I write, I do so based on what I have read in medical journals.  I present what's established by the medical community.

I was an inquisitive child and I am an inquisitive adult, therefore I am always asking the question "why."  When my son was diagnosed in utero with Hypoplastic Left Heart Syndrome, I had A Lot of questions.  Four repairs later (Arch repair, Norwood, Glenn, Fontan) I still have questions.  Why the Arch repair? Our doctors thought Carter had a chance at being two ventricle. Why it didn't work and he had to become single ventricle? It just didn't work for him. Some questions are easier to answer.

Over the past seven years I have met many heart families. An amazing bunch you all are I've learned. Some of their children are doing great, some struggle, and some we have lost. That makes me ask WHY??  Some questions are harder to answer.

My son has HLHS. The future is bright, but there will always be questions.

So that's why I write this blog.

And here's my disclaimer:

I am not a doctor; I do not give medical advice. I'm just a mom of a heart kid who will always have questions. I hope that through my asking questions I can answer some of yours too.

Julie

New Research Survey for Post-Fontan Patients!

The University of Michigan C.S. Mott Children’s Hospital has an important Research Study for children who have completed the Fontan (third stage) surgery. This research study is to evaluate Protein Losing Enteropathy (PLE), and Plastic Bronchitis (PB) in post-Fontan patients. You are invited to complete this anonymous survey whether your child has or has not been diagnosed with PLE or PB. This information will help the doctors learn more about PLE and Plastic Bronchitis and find a way to successfully treat these complications. 

Complete the Survey!

 

Meet Chelsea!

About a month ago, I had the pleasure of meeting a very sweet young lady through the HLHS-Hope Facebook page by the name of Chelsea Schnell.  Chelsea is an HLHS survivor, age 19. She is a college student, athlete, fashionista, and blogger.  Chelsea was kind enough to answer what I think are common questions asked by parents of children with HLHS.  Chelsea began her own blog to help others with HLHS. Please visit Smile, life is beautiful.   

Q&A:

How old were you when your parents first explained to you that you had a heart defect? Do you remember what they said?

I am sure that they had told me before but the first time I really understood that I was a little bit different, but wasn’t till elementary school that I got the “heart talk”. I remember my mother saying “Now remember you aren’t like everyone else, you cannot push yourself. When you are tired, you are tired so stop and take a break.” My parents never said you have a heart problem so you cannot do this or that. We never talked about it; there was no reason to when I was young because I was never really affected by it. I get told more about my heart now than I did when I was a child.

Do you remember if when you were in elementary school you had a hard time keeping up with your peers? How did you handle this?

No I never had a hard time. I ran and played at recess just like everyone else. I never felt different during physical activity. Elementary school was easy, Middle school is when it got harder because that’s when people realized I was the only one with a scar.

Do you feel different from other kids because you have HLHS?

No I don’t feel any different; I just know that I must be healthier than most teens.

Do you ride on roller coasters?

Yes! Of course I do, I love heights and scary rides. There is not a ride I won’t do!

Do you play sports?

I love sports! The first sport I played was T- ball when I was 5, then I started figure skating when I was 6 and still continue to skate. Throughout my sports career I have played basketball for 6 years, I rode motor cross for several, I threw shot put and ran track in middle school and high school I was captain of the Varsity Tennis team.

Do you take any heart medications? Do you feel any side effects because of your medications?

For my heart I am on Linsinopril which I take every day. Along with that I take Actigall, folic acid, baby aspirin (3 days a week) and Centrum vitamins and Caltratep. I do not feel the side effects from the medicine but if I miss a day then I feel side effects which are tiredness and lack of energy.

What has your doctor said to you about your heart in the future? Will you need any other surgeries?

No, no surgeries are in my future and thank the Lord! I am different than most cases of HLHS when I was born all of my stats were in the 95-98% it was like I never even had heart surgeries or a half a heart. I am so blessed to say that my future looks just as bright as any other persons. My future is up to me and how I live. I must take care of myself and know what’s good for me and what is not!

What has your doctor said to you about drugs, smoking and alcohol?

NO, NO & NO! Drugs absolutely not, I even try to stay away from Tylenol and Advil. Smoking is not even something to consider. I do go to college and of course I go to parties and out with my friends to events, but if someone is smoking I walk away or leave plus it makes me feel so sick if I do inhale it. Alcohol; never had a sip or even a finger dip. I do not want to risk it. Alcohol is just as bad as caffeine for HLHS babies. I was told to stay away from all soda drinks, coffee, teas and absolutely no energy drinks. I drink tons of water since the only way my blood travels throughout my body is by pure gravity, the water helps pump my blood around faster and better since my heart cannot!

What have you been told about birth control and having children in the future?

This is probably the toughest part about having HLHS, I could go through numerous surgeries, doctors’ visits, stress test, IV’s, anything but being told I could not have children was the hardest and most painful. I always wanted to be a mother since I was young, I wanted triplet girls and then to adopt, my mother thinks I am crazy but that is what I want. I cannot have children of my own the doctor said but there are ways around it. Surrogacy is most likely what I am going to do and is the best and really only option other than adoption. But see also I use to pray to God when I was little that he would let me adopt a little girl from Asia, well God granted my wishes. So me not being able to have children of my own leaves me to adopt a daughter I have always wanted.

Birth control has also been a sticky situation. I cannot go on the pill or shot or patch because I cannot have estrogen in my blood stream. I can get an IUD which is what most HLHS women have. But my mother is always worried that is it not 100% guaranteed protection. My Gyno is working on finding me something that is easy just like the pill; she is still working on that. My Gyno said the safest and most practical thing to do is get my tubes tied. I am contemplating that and am pretty sure I will go and get the surgery for it.

It is just a risky situation altogether because if you cannot use birth control, then if you do get pregnant your heart is not strong enough to support both you and the baby but you also cannot even think about abortion because your heart cannot handle the medicine from an abortion.

Protein Losing Enteropathy: A Post-Fontan Complication

As defined by PubMed Health, Protein Losing Enteropathy (PLE) is an abnormal loss of serum protein from the digestive tract or the inability of the digestive tract to absorb proteins. PLE is a rare, but well-known complication of HLHS, which may develop in some children weeks or years after the Fontan procedure. Symptoms include swelling in the abdomen and feet, diarrhea, fever, and abdominal pain. Complications include malabsorption due to diarrhea, an impaired immune system which increases the chances of infection, and low cardiac output.

Sadly, PLE is associated with an alarming mortality rate. The 2007 study, Protein Losing Enteropathy after Fontan Surgery - Clinical and Diagnostical Aspects by Tarnok, Bocsi, Lenz, and Janousek, from the Department of Pediatric Cardiology, Cardiac Center Leipzig GmbH, University of Leipzig, Germany, states that between 2,000 and 5,000 children have been diagnosed with PLE. Of the children that have major cardiac surgery, about 7-10% becomes afflicted with PLE. Of these, there is only a 50% survival rate.

 

The cause of PLE in post-Fontan children is unknown. There are many studies on this condition suggesting the use of various medications such as steroids and diuretics for treatment, but further research is necessary to better understand this condition. 

Currently, the Children’s Hospital of Philadelphia, through its Single Ventricle Survivorship Program, follows a treatment protocol that has proven effective in managing PLE. The use of Sildenafil (Viagra®, Revatio®) lowers pulmonary flow to improve cardiac output after the Fontan operation, in addition to the use of the steroid Entocort, to reduce bowel inflammation. 

Talk to your child’s doctor to learn more about PLE and the symptoms of this condition.  

If your child has been diagnosed with PLE and you are interested in connecting with a support group, please contact the Congenital Heart Information Network for more information. 

Current Research Studies of the Top Ten Best Children’s Heart Hospitals

As a parent of a child with HLHS, I often think about what concerns other heart parents have about their children. Although our children have a common diagnosis, they are very different developmentally, physically, socially, and emotionally. A general internet search of Hypoplastic Left Heart Syndrome leads us to basic information on the diagnosis, heart anatomy, surgeries and transplant and very little else, but leaves us with many questions. How does a child develop HLHS? Can HLHS be prevented? What does this mean to a child with HLHS? What does this mean to a family of HLHS?  How best to share our information with one another? How can we help the doctors put the pieces together of this puzzling heart defect? One goal of mine is to find ways to expand on the current published knowledge parents can access regarding HLHS. I also strongly encourage parents to participate in research studies involving our special kids. This is the key to answering the many questions we have about HLHS.  In the meanwhile, I would like to share with you some of the current research studies the Top Ten Children’s Heart Hospitals are currently working on to better care for our children.

Children’s Hospital Boston

Principal Investigator: Jane Newburger, MD
Protocol Title: Brain Structure and Function in Adolescents after the Fontan Operation
http://www.childrenshospital.org/research/clinical/TrialDescr.cfm?id=236

Principal Investigator: Pediatric Heart Network Multi-Center Study
Protocol Title: Single Ventricle Reconstruction Study Extension

http://www.pediatricheartnetwork.com/svrextensionforparents.asp

Principal Investigator: Pediatric Heart Network Multi-Center Study   
Protocol Title:  Fontan Follow-Up Study Basics

http://www.pediatricheartnetwork.com/fontan2forparents.asp

Principal Investigator:  Mark C Wesley, MD   
Protocol Title:  Pharmacokinetics of Tranexamic Acid (TXA) in Children Undergoing Repair of Congenital Heart Defects Utilizing Cardiopulmonary Bypass

http://www.clinicaltrials.gov/ct2/show/NCT01045356?term=Congenital+heart+defect&recr=Open&rank=6

Principal Investigator: Michael Agus, MD  
Protocol Title: SPECS: Safe Pediatric Euglycemia in Cardiac Surgery 

http://www.clinicaltrials.gov/ct2/show/NCT00443599?term=Congenital+heart+defect&recr=Open&rank=22

Principal Investigator: John M Costello, MD MPH
Protocol Title: Effects of Perioperative Nesiritide or Milrinone Infusion on Recovery From Fontan Surgery 

http://www.clinicaltrials.gov/ct2/show/NCT00543309?term=Congenital+heart+defect&recr=Open&rank=34

The Children’s Hospital of Philadelphia

Principal Investigator: Jack Rychik, MD
Protocol Title: Sildenafil After the Fontan Operation (SAFO) 

http://clinicaltrials.gov/ct2/show/NCT00507819

Principal Investigator: Robert E Shaddy, MD   
Protocol Title: Evaluation of Panel Reactive Antibody in Children Following Stage I Palliation for Hypoplastic Left Heart Syndrome

http://www.clinicaltrials.gov/ct2/show/NCT01135485?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3APA&age=0&rank=4

Principal Investigator: Felice Su, MD
Protocol Title:  Understanding Dexmedetomidine in Neonates After Open Heart Surgery

http://www.clinicaltrials.gov/ct2/show/NCT00576381?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3APA&age=0&rank=8

U-M C.S. Mott Children’s Hospital

Principal Investigator: John Charpie, MD, PhD    
Protocol Title: SPECS: Safe Pediatric Euglycemia in Cardiac Surgery

 http://www.clinicaltrials.gov/ct2/show/NCT00443599?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AMI&age=0&rank=1

Texas Children’s Hospital

Principal Investigator: Tia Tortoriello Raymond, M.D.   
Protocol Title: Trial of Vasopressin and Epinephrine to Epinephrine Only for In-Hospital Pediatric Cardiopulmonary Resuscitation

http://www.clinicaltrials.gov/ct2/show/NCT00628550?term=heart&recr=Open&state1=NA%3AUS%3ATX&age=0&rank=12

Lucile Packard Children’s Hospital

Principal Investigator: Stephen J Roth, MD, MPH  
Protocol Title: Aminophylline to Prevent Acute Kidney Injury in Children After Cardiac Surgery (KID PRO AM).

http://www.clinicaltrials.gov/ct2/show/NCT01245595?term=Congenital+heart+defect&recr=Open&rank=27  

Children’s Healthcare of Atlanta Pediatric Hospital

Principal Investigator:  Brian Kogon, MD
Protocol Title: SPY Imaging System: It’s Role in Pediatric Cardiac Surgery 

http://www.clinicaltrials.gov/ct2/show/NCT00446927?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AGA&age=0&rank=1

Principal Investigator: Shannon E Hamrick, MD      
Protocol Title: Magnetic Resonance Imaging (MRI) to Evaluate Brain Injury in Congenital Heart Disease (CHD Brain) 

http://www.clinicaltrials.gov/ct2/show/NCT00932633?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AGA&age=0&rank=4

Principal Investigator: Denver Sallee, MD  
Protocol Title: Abnormal 3-D MRI Flow Patterns in Adolescents Patients with Bicuspid Aortic Valve

http://www.clinicaltrials.gov/ct2/show/NCT00412386?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AGA&age=0&rank=6

Children’s Hospital of Wisconsin

Principal Investigator: Steve Zangwill, MD      
Protocol Title: Erythropoietin and Pediatric Cardiac Surgery (EPO) 

http://www.clinicaltrials.gov/ct2/show/NCT00451698?cond=congenital+heart+defect&state1=NA%3AUS%3AWI&age=0&rank=1

Principal Investigator:  Rohit Rao, MD  
Protocol Title: Multi-Site Near Infrared Spectroscopy (NIRS) Monitoring of Children During Exercise 

http://www.clinicaltrials.gov/ct2/show/NCT00556231?cond=congenital+heart+defect&state1=NA%3AUS%3AWI&age=0&rank=2

Principal Investigator: Nancy Ghanayem, MD  
Protocol Title:  Near-Infrared Spectroscopy (NIRS) Neurodevelopmental Outcomes (NIRS ND)

http://www.clinicaltrials.gov/ct2/show/NCT00464100?cond=congenital+heart+defect&state1=NA%3AUS%3AWI&age=0&rank=5

Cincinnati Children’s Hospital Medical Center

Principal Investigator: Katherine Krawczeski, MD     
Protocol Title: Intravenous L-Citrulline to Treat Children Undergoing Heart Bypass Surgery 

http://www.clinicaltrials.gov/ct2/show/NCT01120964?term=citrulline&recr=Open&state1=NA%3AUS%3AOH&age=0&rank=1

Nationwide Children’s Hospital

Principal Investigator: Vidu Garg, MD
Protocol Title:  Genetics of Congenital Heart Disease

http://www.clinicaltrials.gov/ct2/show/NCT01192048?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AOH&age=0&rank=1

Principal Investigator: Aymen N Naguib, MD           
Protocol Title:  Measures to Lower the Stress Response in Pediatric Cardiac Surgery

http://www.clinicaltrials.gov/ct2/show/NCT00848393?recr=Open&cond=congenital+heart+defect&state1=NA%3AUS%3AOH&age=0&rank=2