Every year, 100,000 babies don't make it to their first birthday because of CHD.
CHD, CHD awareness

Steven Lee Burns is our heart Warrior of the week! He was born on March 16th, 1990 with hypoplastic right ventricle and pulmonary atresia with an intact ventricular septum.  Steven said his parents had no clue about his heart defects going into his birth. In fact, early on in his mom’s pregnancy, her body tried to terminate the pregnancy but her doctor’s were able to prevent it.

Throughout his journey with CHD, Steven has undergone an astonishing 7 open-heart surgeries and countless catheterizations. In his first few surgeries, he had a BT shunt inserted, as well as a right ventricular outflow patch/conduit placed to bypass the defective pulmonary valve.  Doctors enlarged the conduit/patch every few years as he grew up until just after his 25th birthday.  During his most recent surgery in April of 2015, they tried a more permanent fix and inserted a pulmonary valve homograft.

When asked how much time he has spent in the hospital throughout his battle with congenital heart disease, Steven said, “That’s actually a good question. When I was a baby I spent most of my years in and out of the hospital before the age of 5.  I would visit the cardiologist every 6 months to a year, and now I frequent the cardiologist as we are trying to form a game plan.  In total, out of my 28 years, I spent a good 7 years in the hospital.” He is currently on three medications that he believes will be a lifelong part of his treatment plan.

Speaking of his treatment plan, Steven was initially misdiagnosed with TOF and did not undergo the Glenn/Fontan. Doctors hoped his right ventricle would keep up with his body, but unfortunately that hope was futile; as he is now sick again.  He is exploring the possibility of a transplant because the right side of his heart can no longer keep up.

Steven, who describes himself as caring, driven, aware, friendly and open, says the following about the challenges and restrictions he faces due to his heart condition:

I have always been told to go at my own pace.  Since my condition is a “cyanotic” CHD, I tire easily and run out of oxygen often.  I am also prone to right coronary fistuals; while playing baseball at the age of 7, a fistula branching off of my right coronary artery drained a great portion of my blood back into the ventricle and caused me to have a “pseudo-heart attack”.  I was rushed by ambulance then by helicopter to receive immediate care.  I don’t really remember much, but it felt like my chest was being crushed by a stack of weights, and I could barely breathe.  I remember hearing the EMT screaming my name, telling me to hang tight, and to keep talking to her.  The last thing I remember was hearing her say, we need epinephrine now.

Living with a CHD also slowed me down socially because I always had to be guarded and was worried about getting sick.  I’ve grown out of that but it definitely impacted my childhood.  I just hope that social media continues to grow and support upcoming CHD warriors and their families.  If I had that growing up, I would have been able to make more friends who understood what I was going through.

I try to live my life day by day, but of course I worry every time I get sick or if my chest hurts, which is why I cherish my friends and family. Despite the worry, I do my best to live every single day to the fullest.

About Steven’s Heart

Hypoplastic Right Ventricle

Anatomy: The right ventricle is either too small or absent. The most common heart problem that includes a hypoplastic right ventricle is called tricuspid atresia.

Complications: Blood can't flow appropriately through the heart and into the lungs to pick up oxygen as it normally would. The result is that the lungs can't supply the rest of the body with the oxygen it needs.

Symptoms: Blue in color (cyanosis), difficulty breathing, tiring easily, especially during feedings, slow growth, poor weight gain, fatigue and weakness, shortness of breath, swelling in the legs, ankles,  feet or abdomen, sudden weight gain from fluid retention.

Treatment: Treatment for tricuspid atresia involves open-heart surgery to ensure adequate blood flow through the heart and into the lungs, allowing the body to receive the proper amount of oxygen-rich blood. Often, this requires more than one surgical procedure. Medications also may be given before surgery.

Prevalence: This CHD occurs in 1 out of every 10,000 live births.

Pulmonary Atresia

Anatomy: Pulmonary atresia is when this pulmonary valve didn’t form at all, instead a valve with three leaflets that open and close, a solid sheet of tissue forms and no blood can go from the right ventricle of the heart to the lungs.

Pulmonary atresia with an intact ventricular septum: the wall between the ventricles remains complete and intact. During pregnancy when the heart is developing, very little blood flows into or out of the right ventricle (RV), and therefore the RV doesn’t fully develop and remains very small. If the RV is under-developed, the heart can have problems pumping blood to the lungs and the body. The artery which usually carries blood out of the right ventricle, the main pulmonary artery (MPA), remains very small, since the pulmonary valve (PV) doesn’t form.

Complications: This anatomy cannot support life because blood can’t flow to the lungs to become oxygenated and deoxygenated blood cannot support normal growth and function.

Symptoms: Abdominal bloating, bluish color to the skin (cyanosis), chest pain, fainting, fatigue, poor weight gain or failure to thrive in infants with severe blockage, and shortness of breath.

Treatment: Depending on the severity of the condition there are a variety of treatment options, including medications, balloon catheterizations, and valve replacement via open-heart surgery.

Prevalence: Pulmonary atresia with a VSD accounts for 2.5-3.4% of all congenital cardiac malformations with a prevalence of 7 per 100,000 live births.

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