Congenital Heart Defect--Pulmonary Atresia

Congenital Heart Defect Facts and Information

The word "congenital" means existing at birth. The terms "congenital heart defect" and "congenital heart disease" are often used to mean the same thing, but "defect" is more accurate. (http://www.heart.org)

Congenital heart defects are structural problems with the heart present at birth. They result when a mishap occurs during heart development soon after conception...Defects range in severity from simple problems, such as "holes" between chambers of the heart, to very severe malformations, such as complete absence of one or more chambers or valves.  Having a congenital heart defect can also increase your risk of developing certain medical conditions. (http://www.heart.org)

Nearly 1 in 100 babies (about 1 percent or 40,000 babies) is born with a heart defect in the United States each year. About 4,800 babies each year are born with CCHD. Many heart defects don’t need treatment or can be fixed easily. But some, like CCHD, can cause serious health problems or death. (http://www.marchofdimes.com/baby/congenital-heart-defects.aspx) CCHD=Critical Congenital Heart Defect

Anyone can have a child with a congenital heart defect...If you or other family members have already had a baby with a heart defect, your risk of having a baby with heart disease may be higher.  (http://www.heart.org)

CHD Awareness Week (FEB. 7-14)is an annual awareness effort to help educate the public about Congenital Heart Defects. Participants include individuals, local support groups, national and local organizations and congenital cardiology centers throughout the world! (http://tchin.org/aware/)

Heart defects can affect your baby’s blood flow, causing blood to:

• Slow down
• Go in the wrong direction or to the wrong place
• Be blocked completely  (http://www.marchofdimes.com/baby/congenital-heart-defects.asp

CHD research is drastically under funded in both the public and private sectors relative to the number of lives the disease affects. In the private sector, only 1% of every dollar received at the major private funder of cardiovascular research goes to any Pediatric cardiac research. Likewise in 2007, the National Institutes of Health's NHLBI (National Heart, Lung and Blood Institute) allocated less than 3% of every dollar invested in research to any Pediatric cardiovascular research.(http://www.childrensheartfoundation.org/advocacy/chd-research)

There are more than 40 different types of congenital heart defects. Little is known about the cause of most of them. There is no known prevention or cure for any of them. (http://www.childrensheartfoundation.org/advocacy/chd-research)

Despite the fact that CHD affects approximately 1.8 millions families in the U.S.,  a relatively small amount of funding is currently available for parent/patient educational services, research, and support.  (http://tchin.org/aware/)

For more Data and Statistics on CHD, see: http://www.cdc.gov/ncbddd/heartdefects/data.html

Here is a great description of our son's defect and the unifocalization surgery he had.  The specialists at Stanford Children's (LPCH) are the very best.

http://www.stanfordchildrens.org/sites/en/service/heart-center/unifocalization.page


This is our story:

Our son Bogey, (one of our twins) was diagnosed with a Congenital Heart Defect--Pulmonary Atresia with VSD & MAPCA's, and Tetrology of Fallot.

THE DIAGNOSIS:
Bogey was born on April 6, 2012 in Spokane WA.  He had been pre-prenatally diagnosed with a cleft lip, and cleft palate. Because he was a twin, and was transverse, he was born via C-section at 35 weeks, 6 days.  He was 6 lbs. 12 oz.  (His twin brother was born a minute later.  He was 6 pounds, 5 ounces.)

It was evident that something was amiss with Bogey because his breathing was rapid and shallow.  He was immediately put under an oxygen tent.  His saturation improved, but his breathing rhythm did not.   After almost two days of observation in the NICU, Bogey underwent an echo cardiogram.

Dr. Pamela Burg was the pediatric cardiologist that introduced us to our son's congenital heart defect (CHD).  She did her best to explain what was happening inside Bogey's tiny body.  Of course, as parents, we were overwhelmed with questions, confusion, and worry.

Because of the combination of the CHD, and the clefts, tests needed to be completed to rule out  DiGeorge Syndrome (or 22q11.2). Genetics testing came back negative.  

PRIMARY CHILDREN'S HOSPITAL After being released from Sacred Heart Hospital, our little family packed up and moved closer to our siblings and parents.  Bogey had an appointment  at Primary Children's Hospital in Salt Lake City, UT for a crainialfacial/plastic surgery consultation.  While we were there, we also met with a cardiologist who took a moment to check Bogey.  She did some blood work and discovered that Bogey's electrolyte levels were extremely off, and he was immediately admitted to the Cardiac ICU.   

It took 10 days for his levels to return to normal range.  

We were released, under the condition that we see our local pediatrician about every other day. 

Ten days later, we received a phone call, notifying us that we were scheduled for surgery at the end of the week.  We were on a plane to Palo Alto CA the following day. 

 

LUCILE PACKARD CHILDREN'S HOSPITAL AT STANFORD Before his surgery, he had an infection that caused him to have an uncontrollable cough.  He ended up "code blue" and in the CVICU at Lucile Packard Children's Hospital the day before he was to have the unifocalization surgery. He was intubated, and put on a paralytic medication, and 14 different medication IV's, to help him beat the illness.  His lung collapsed, he fevered, and was just plain sick.  Bogey's medical team decided it would not be safe for him undergo surgery.  

We waited just over a week for Bogey to be strong enough, and over the infection enough, to endure open heart surgery.  He was  2months old--exactly.  He entered the operating room at about 8:00 a.m. and returned to the CVICU at 7:00 p.m.  It was long day.  Dr. Frank Hanley did a complete repair (unifocalization).

The recovery was long and hard.  Bogey had great days, but he also a lot of bad days.

During this scary and "foggy" time in our lives, we were so blessed to be at such a great hospital with exceptional nurses and educated doctors that took such special care of our little baby.


BACK TO PRIMARY CHILDREN'S HOSPITAL 
Three days after we were discharged from our 6 week stay at LPCH, we were at home, and Bogey was having a hard time breathing, he was sweaty, and lethargic.   We knew something was wrong.  We took him to the E.R. and because his condition is so rare, he was on a Life Flight back to Primary Children's Hospital.  It was soon discovered that he was in respiratory and right heart failure.  His lung collapsed again, he was intubated, and put back on his pre-surgery medication.  

We don't know what might have caused the failure--possibly higher elevation--but it took 4 more weeks for Bogey to be well enough to come home.  He was on supplemental oxygen, and diuretics.  Little by little, he got stronger.  After 3 months, he was off the supplemental oxygen, and down to only one diuretic medication.  At that time, he underwent his cleft lip repair, and 5 months after that, a cleft palate repair.

Our little boy has needed occupational therapy, physical therapy, and will probably need speech therapy too.  He is making progress.  He has a g-tube, (it was inserted when he was 3 weeks old, sucking from a bottle was much too difficult for him) and we are currently trying to eat enough orally to gain weight, so that we can get the tube removed.  It's much harder than I had anticipated.

This 14 minute video shows pictures before and after the surgery.

We are definitely not experts on this defect, but we're learning.  Everyone has different experiences with illness, but I wanted to post about our son and his defect so that we could reach out to others who have similar conditions, in some way.  

If you want to see more or read more about Bogey and his hospital stay(s), you are welcome to browse our Blog Archive, starting in April 2012.  Or, you can contact us via e-mail:  jrpar5@yahoo.com 

*UPDATE** UPDATE**UPDATE**UPDATE**UPDATE**UPDATE**UPDATE**UPDATE*

I just wanted to put in an update on our journey with this CHD.  On September 25, 2015 our son (3 years old) had his conduit replacement.  It was his second open heart surgery.  We returned to Dr. Hanley for the procedure.  This surgery went so much smoother than the first.  The recovery was fairly quick, and easy.  We were discharged with 1/2 children's asprin per day, as well as lasix.  After one month, we were off the lasix. 

If you want a more in depth report on our trip to Stanford Children's (Lucile Packard) Hospital, you can read about it here:  http://wilhelmsenpar5.blogspot.com/2015/10/ohs.html