Second Opinions: It Never Hurts

The following letter arrived in the CHD Blog's inbox from Farshid Bayat...

My daughter Ava was born with DORV, D-TGV, PDA, large VSD, ASD and COA on 21 October 2006. In a nutshell, Ava had two surgeries at the ages of 2 and 12 months. Her final surgery took place on 27 April 2008. Although most of the cardiac pediatricians we had visited before were of the opinion that Ava's life could not be saved, the excellent pediatric cardiac surgeon Dr. Navabi Shirazi managed to thoroughly repair the main defects in a ten-hour surgery. Ava was discharged from the hospital after a ten-day stay.

Today Ava acts like any other child with no physical restrictions. She is a bundle of energy who loves running and playing football with her older friends. Parents of children with CHD should remember to ask for a second opinion before choosing a surgeon. Had we known about Dr. Navabi from the very beginning, Ava would have been treated by only one surgery.

I wish I could say that it's the only time I've heard of such a story, but it simply is not the case. Until we have a central registry of CHD cases and better reporting information for CHD cardiac care facilities, the need for second opinions will remain vital in your (or your loved one's) quality care. Many times, at diagnosis, we have little time to prepare. We may feel comfortable with the individual(s) who provide the diagnostic results and recommendations, and be lulled into a sense of comfort in working with them. But I urge patients and parents alike to be diligent - ask those tough questions, research the facility and its staff carefully and talk to fellow CHD survivors and families when you can - it can make a tremendous difference.

Stem Cells May Help Repair Valve Defects!

Bloomberg Science, Nov. 10 -- Cardiologists at the University Hospital of Munich believe that umbilical cord blood, rich in stem cells, may bre the raw material needed to repair the hearts of thousands of children born each year with defective heart valves. The researchers reported the findings at the November 10 annual meeting of the American Heart Association. They believe they are 5 to 7 years away from transplanting new valves created from cord blood with patient's faulty valves.

The Munich researchers are ready to begin trials in lambs, using collected cells (frozen for preservation) to seed into biodegradable polymer scaffolds. Thus far, eight bio-engineered valves have acted much like natural heart valves when tested for blood flow and pressure. The scaffolds will disolve over time, leaving behind a fully formed structure made from the cells, they assert. The next step will be to transplant the heart valves into hearts of young lambs, watching them to see how they grow and function over time. The experiments are hoped to begin next year.

Click here to read more...

Information from US NIH

I found a fabulous cross-referenced website I'd been missing last night - the National Institute of Health (US) has a webpage chocked full of information and news on CHD. Check it out!

This has been the most helpful page I've found so far... they have links to videos, clinical trials, current research, recent news and advancements, treatment options and more.

Why is it so hard to find info on CHD? Maybe I just wasn't looking in the right places before...

7 For 7: 7 CHD Stories

Today's 7 For 7, features just a few of the stories that have been shared with me by my newfound CHD friends, through this blog, or online. You will not stand up from reading this post without your heart feeling lighter!

Cynthia's Story. I am a 60 year old survivor of Tetralogy of Fallot. I had the Blalock-Taussig Shunt when I was 3 and a total correction at 15 years. In 2004, I had to have my pulmonary valve replaced. In addition, an aneurysm had formed in the teflon that was used to expand the pulmonary artery during the total correction. This was also repaired in 2004.

Throughout my life, I've always worked to overcome shortness of breath, becoming tired, and all of the other symptoms that go along with this defect. I realize that I push myself further than anyone else pushes me.

I have discovered an organization, the Adult Congential Heart Association, that I would like to share with you. People like me who have lived longer than anyone expected them to live can present a problem to doctors. We're sort of leading the way for the children who are just being born with CHD. By the time some of these children reach my age, I hope that the things that are learned now in managing my health and the health of other adults will be standard procedure for the management of their health.

Parents, prayers are answered every day. As you all have indicated, your children are already a blessing and, in many ways, are blessed. I would not have lived if Dr. Blalock and Mr. Vivian Thomas had not worked diligently for years to develop the surgery that saved my life. Others are working equally as hard to find ways to save the lives of children born today and tomorrow. My thoughts and prayers are with all of you and all of the children as you move through life.



Misty's Story. My CHD story begins on New Year’s Day, 2005 when I found out I was pregnant with my 3^rd child. My pregnancy was pretty normal. All of my ultrasounds were fine and there was no cause for concern, so we thought. Sydney was born on August 24, 2005. Her APGAR scores were 9 and 9. Everything looked good until the pediatrician came in and checked her out and heard a murmur that was “slightly louder than what we like to hear” and called in a pediatric cardiologist, Dr. Albrecht.

At 4:30 pm that afternoon, Dr. Albrecht came into my room with a nurse. I was there by myself taking a nap and waiting for my newest little girl to be able to come to my room and get to hold her. I’ll never forget what he said. “Your daughter has a problem with one of the valves in her heart and I have called a transport team from UVA Medical Center to come and pick her up and transport her there. She will have surgery tomorrow morning.” Sydney has Aortic Stenosis (AS). Dr. Albrecht advised me that her Aortic Valve was a bi-cuspid, instead of a tri-cuspid, valve and was barely functioning and letting very little blood out of the valve to the Aorta. After he went over everything, he sent the nurse to bring Sydney to my room so I could say my “hello’s and goodbye’s”. My husband called his parents to go pick up our other 2 children and bring them to the hospital to meet their new little sister before they took her an hour away to prepare her for the surgery the following morning.

The next morning my husband called to let me know that they were getting ready to take her back and that she had so many tubes and wires coming out of her that the only place that you could touch her was on her head. At that point, he started to break down, again, and I began falling apart, again. Two hours into her procedure, we received the news that everything went well, she had had two blood transfusions, and she was going to be ok.

When I was finally released from the hospital and able to go see my daughter two days after her birth, I found her in her little bed with an intubation tube and many tubes and wires attached to her. Since that day was my birthday, Steve’s parents brought our two older children up to the hospital to see their sister. I didn’t want them to see her with all of the tubes and wires, but someone brought them back before I could say anything. What was amazing was that when she heard them start talking, her heart rate stabilized, her respiratory rate normalized and her blood pressure returned to a normal state. It was incredible.

Today my daughter looks like a normal 23 month old, but she is not a normal little girl. There are things that she will have to go through that ‘normal kids’ won’t have to do. Besides the future surgery(ies) to open and eventually replace her aortic valve, she won’t be able to do some sports that she may want to do and she will, most likely, have to take medicine every day of her life. Her heart will never be normal.

Joshua's Story. Joshua is an active 4 year old. He was born in June of 2003 with hypoplastic left heart syndrome, a rare and fatal defect, unless treated. Basically, he was missing his left ventricle and his aorta was very narrow. Parents of children with this defect are given three options: they can take their child home to die, they can try for a heart transplant in hopes that a heart will become available soon enough, or their child can undergo three surgeries that will reroute the blood so that the child can survive. Jodi and Mark, Joshua's parents, opted for the surgeries.

Joshua developed severe blood infections after his first surgery, but miraculously pulled through, and his parents were able to bring him home almost two months later. His second open-heart surgery took place when he was 5 ½ months old, and he went home five days afterwards. He had his third surgery, the Fontan, when he was three. This surgery was particularly hard on the family as they had had over two more years to learn to love Joshua, and they knew there was a possibility he might not come home. However, Joshua also made it through this surgery with a few post-surgery complications called pleural effusions. Though it kept him in the hospital longer than anticipated, Joshua did fully recover.

We do not know what is in Joshua's future. The doctors cannot "fix" his heart. We are hoping that Joshua will not need any other procedures for a long time. In the meantime, he has fun playing with his brothers and making his parents laugh. Every day with him is a blessing.

Sharon's Story. Saving the life of infants us everyday work for Dr. Karen S. Rhueban, a Pediatric Cardiologist at University of Virginia Hospital. She was interrupted from an interview to perform an emergency cardiac catheturization on a newborn 4 1/2 pound twin whose blue-tinged skin indicated her blood was not circulating properly to receive oxygen.

In the cardiac catheturization laboratory, Dr. Rheuban inserted a plastic tube in the baby's leg and laced it through blood vessels to the heart to record circulation of the blood and make an accurate cardiac diagnosis. The end of the tubing was blown into a nickel-sized balloon and passed through a hole in the upper chamber of the baby's heart to allow blood to circulate. Known as a balloon atrial septostomy, this procedure saved her life. The baby had to have open-heart surgery at 9 months old in order to survive.

Today, Sharon is 23, and a marine wife. She leads a normal life with a daughter of her own, who has a clean bill of health. Two adults have told me in the last week that they had never met any one else with CHD. Sharon is one of them!

Christy D' s Story. My husband were excited when we found out I was pregnant with my second child. Then our world came crumbling down at our 21 week ultrasound; Steve and I were told our little baby girl had a severe and life threatening heart condition. Instead of joy, we were expecting, our hearts were filled with pain, fear and grief. The baby was diagnosed with Tricuspid Valve Atresia (TA)---meaning her tricuspid valve did not form. Without her tricuspid value, her blood was unable to flow into her right ventricle, enabling growth; meaning she has a 3-chambered heart.

Sydney was born on December 4th, 2006 and had surgery 8 days later. Without this surgery, which has its own risks, she would not survive. The recovery was a roller coaster ride. Sydney developed seizures 1-2 days post-op, which is a risk to having open heart surgery. Her O2 sats, heart rates, and blood pressures were all over the place. She had so many tubes and lines in her, that the nurses had run out of places to put them. After many prayers and medical assistance, we got to take Sydney home just 3 weeks later, just in time for Christmas. What a great gift!!!

At 4 months of age, Sydney had her 2nd surgery. Just remembering Sydney going through surgery last time, made us sick with grief. We knew she was in the best hands, but there are never any guarantees when it comes to surgery. Thanks to all the Angels above, the 2nd surgery was a breeze compared to the first surgery. After the surgery, Sydney's life has improved in a dramatic way. At approximately 2 ½ years of age, Sydney will have her 3rd surgery, as long as everything continues to progress as planned.

Sydney, despite her medical problems, amazes me. She is full of love and life. She always has a smile, no matter what is going on. I thank God for her every day, and despite the fact it is hard being a “heart parent”, there is NEVER a day, or a moment, I would change it, if it meant not having Sydney in our lives.

Karen's Story. As I lay on the exam table and the ultra sound tech squeezes some warm gel on my expanding belly, I look over at my two daughters who are 4 and 7 and squeeze my husband’s hand. We are all eager to find out the sex of our newest addition to the family. With two girls already, we were eager to find out if Daddy will get a little boy. The baby is uncooperative. The tech is unable to tell the sex. Our excitement elevates. The tech steps out to get the doctor. Soon the doctor comes back. He looks at the screen for a long time. I start to get a sick feeling in my stomach. I squeeze my husband’s hand harder. The sweat is building between our tightly gripped hands. His panic is beginning to flow to me threw his hand as though our bodies were connected. The doctor speaks…. “This is not good. This is bad.” I truly feel like I am going to faint. I look over at my girls who have started to look at a Dr Seuss book and have forgotten about what we are there for. I think to myself that they understand “This is bad.” Get them out of here. I don’t want them to see me cry. Oh my God! Oh my God! The tech takes my daughters out of the room to the waiting room. The doctor tells us that the baby is missing part of its heart. Missing half it’s heart! What does this mean? Is the baby going to be able to live?

We were sitting in the doctor’s office and the cardiologist began explaining everything. Our baby has Hypoplastic Left Heart Syndrome. To this day, I don’t remember that doctor giving us much, if any, hope. But I don’t remember much from that conversation. The one thing I do remember was we were told to think about aborting the baby. After many tears and prayers we decided to continue with the pregnancy and do everything we could for our baby.

Jameson was born on October 13, 2006. When she was four (4) days old, she had her first open heart surgery. The next couple of months were extremely difficult. Jameson was stable but, by no means, was she a healthy new born. She struggled to eat and thrive. She had her second open-heart surgery at 4 1/2 months. The recovery was much quicker this time. The best part was she was crying, crying because she was hungry and wanted to eat! Her third, and hopefully final surgery will be in April.

Every month Jameson has grown stronger. She is one now and is such a wonderful baby. She sometimes gets winded when she is crawling or moving around but other than that, the scar that is hidden under her shirt is the only proof of what this little girl has been through.

Parents deserve to believe their child has a chance. Had we followed the doctor’s advice we would not have our beautiful “Little Champion.”

Christy H's Story. At 16 weeks pregnant we learned through an ultrasound that our daughter had lung issues. At 23 weeks, we were shocked and devastated to learn of her heart defects. Between the two, she was given a 5% chance of survival. Despite the odds, my daughter, Harlie, was born on September 25, 2006.

Harlie has a CHD called Congenitally Corrected TGA and three other heart defects that complicated matters and forced the surgeon to do a three operation “repair” to re-plumb her circulation. I have been told that her heart will last her about 30+ years and that after that we will be looking at a transplant.

In addition to her heart defects, Harlie also has Goldenhar Syndrome (an asymmetrical craniofacial syndrome). She has an underdeveloped jaw and missing bones, low forward set ears, with a dysmorphic left ear and no canal, many vertebral anomalies, left eye anomalies, misshapen skull, anoperineal fistula, and a lung malformation that required the removal of most of her right lung.

Harlie is now 16 months old and has just started to crawl. She has a 20-word vocabulary in sign language. She breathes through a tube in her throat, called a trach. She eats through a tube that was surgically placed in her stomach. She’s had seven surgeries and has spent over four months in hospitals and she’s just getting started.

Despite all of this, Harlie is amazing! She’s smart and happy and we think that she’s quite cute. She has such an agreeable personality and just seems to go with the flow, whether she’s at home or in the hospital. We are very thankful that we have her.

Harlie's heart, lung, airway and feeding issues are pretty overwhelming. But I try to focus on what’s good and be thankful for those things. I try very hard not to think too much about her heart or her lungs. I see children running and playing everyday and wonder if Harlie will be able to do that. I try very hard not to think about what it will be like when she realizes that she looks different. Or the first time she comes home crying because someone made fun of her. We are a very vain society, and that will make parenting her much more challenging.

But worrying about all that is a waste of energy – energy I need to just get through each day. I do my best. I take very good care of her. I make all the necessary appointments with her 14+ doctors, I got numerous opinions until I found the right doctors with the right plans for her. I give her all her medications as prescribed. I’ve learned CPR and she wears an oxygen and heart rate monitor when she sleeps. But, in the end, despite all my efforts, I cannot control how her heart functions. I just hope that it beats like it’s supposed to and that it doesn’t stop. I hope that she never gets a plug in her trach that blocks her ability to breathe. I hope that she never pulls the trach out (again! – yes that was a close call). I hope I never have to call 911 again. I hope that she will know that her care was never too much for me and that she is worth whatever I have to do to keep her safe and sound. I hope that I can continue to be the strong mother I need to be for her. And I hope that she will know that she has made me the happiest mom on the planet.

7 For 7: 7 CHD Faces and Stories

Today is the first, official day of CHD Awareness Week. This week's blog entries will each feature lists of 7s relating to CHD. We hope you will enjoy it, maybe learn something new and pass it on! Quite a few friends are participating in this effort, and we want to thank them in advance for their stories, photos, shared memories and other information.

Today's entry features 7 beautiful people who have broken, mending or mended hearts as a result of CHD. Each has a unique story and has faced many challenges of CHD. Some have more challenges to face. No two have the same results from CHD, but all have the same common goal - making the most of the heart they have been given!

Jameson is a 16 month old with Hypoplastic Left Heart Syndrome. She is a loving and happy toddler, full of hugs and kisses, and an absolute joy to be around. Her first open heart surgery took place when she was 4 days old, and her second at 4 ½ months. After her second surgery, Jameson began to grow stronger every day. Lately, though, she sometimes gets easily winded when she is cruising around, and her stats are chronically lower than normal. Other than that, the scar that is hidden under her shirt is the only proof of what this little girl has been through. Her third surgery (the Fontan) will be in April.


Lauren was diagnosed with Tetralogy of Fallot at birth. Her parents were told that she probably wouldn't make it and I was taken by ambulance to UNC Chapel Hill where I had open heart surgery at a week old. There they performed a temporary repair that lasted until she was 17. Lauren then had another surgery to close up the shunt and perform a homograph pulmonary valve replacement. Her family and friends have always been very supportive and treated her as an equal to her sister, who has a healthy heart. She now is the co-coordinator of Mended Little Hearts in Central Virginia, and is grateful to have been given the opportunity to live and help others. At age 36, there is always a probability that she will have to have another surgery, but for now she is able to do anything I want to do (except be a flight attendant - she's not tall enough!).

Gabe was born in July 2006 and surprised his family with the news that along with a full head of hair, he also had a broken heart. He was diagnosed with D-Transposition of the Great Arteries, minimal Pulmonary Stenosis, and a bicuspid valve. He has undergone two open heart surgeries to repair his heart, and now has pulmonary stenosis due to the nature of his second repair. His family has high hopes that he will outgrow it and that if any intervention is needed, it will be in the cath lab. His mom says he disobeys her with glee, and is a "blur these days... ever moving!"


Sydney was diagnosed with Tricuspid Valve Atresia (TA), meaning her tricuspid valve did not form. Without it, her blood was unable to flow into her right ventricle, enabling growth, leaving her with a 3 chambered heart. She had her first surgery when she was 8 days old and her second at4 months. At approximately 2 ½ years of age, Sydney will have her 3rd surgery, as long as everything continues to progress as planned. She will never be “fixed” unless a way of creating a new chamber is discovered through further research.

Harlie has Goldenhar Syndrome, VACTERAL Association, and Congenital Lobar Emphysema. Harlie’s heart defects are: L-TGA, VSD, intermittent 2nd degree heart block, small right ventricle, and 2 small Superior Vena Cava’s. She was also born with a mass in her chest, and a variety of other physical anomalies. To date, she has had 7 surgeries and spent over 4 months total in the hospital. Despite everything, she is one of the sweetest and happiest babies you could ever hope to meet. In the past two months she has learned 21 signs, and loves show off her new communication skills, which include putting two words together. She hardly ever cries and is absolutely beautiful - your heart just melts when you meet her!

Claire is currently in the hospital with RSV - send positive energy and prayers her way! She was born with TGA and VSD, as well as a faulty valve, which resulted in her having to have a Rastelli Repair, which uses artificial material inside and outside the heart to create the figure eight necessary for normal oxygenation of the blood. If all goes as expected, Claire should be able to act like any other child. Though she will have to have at least a couple of conduit replacements as she grows, there is a bright side there as well. The conduit replacements are not quite as "invasive" as they are at the front of the heart, research on expanding the conduits without open heart surgery to prolong the time before replacement is taking place and there is the possibility through stem cell research that a conduit could be grown from Claire's own cells that would grow with her.

Sadie came into the world screaming, but quickly turned blue and was taken from her parents. She was diagnosed six hours later, when she was critically ill, with TGA, ASD and VSD. She received a catheturization that night, and took 11 days to stabilize enough for the surgery that was required for her survival. Though she suffered drastically from drug withdrawals after surgery, she quickly healed. Today she is a strong-willed bundle of energy. She will continue to receive annual cardiology check ups, but there is a very good chance that no further invasive procedures will be necessary in her life time.