February 15, 2019 // Perspective
Sudden cardiac death in the young
I was once called, “stat,” to the emergency room to see a 17-year-old young man who collapsed while playing a pickup football game with friends at a local high school. The school day had ended and there were just a few teachers left at the school. Minutes went by before a teacher got to the scene to find the student lying lifelessly. She called 911 and did her best to begin CPR.
When the medics arrived, it had been nearly 10 minutes since he had crumbled to the ground. They took over doing CPR, hooked up the EKG and found his heart was in ventricular fibrillation. On the third shock his heart began to beat again and they could feel a weak pulse. He was not breathing, so he was intubated and bagged vigorously to deliver oxygen to vital organs, but most importantly to the brain.
On my arrival at the emergency room I found his pulse and blood pressure to be getting stronger. As I examined him from head to toe, I reviewed in my mind the differential diagnosis (causes) for such a catastrophic event. He was comatose as we took him up to the intensive care unit.
Days turned into weeks and he remained deeply comatose. The neurologist was not very optimistic and told me there was less than a 5 percent chance of recovery to a functional state and almost no chance of recovery to an independent living status. After six weeks he remained in a coma and was not capable of breathing on his own. Several of the doctors on the case with me suggested it might be time to consider withdrawing his ventilator support.
His mother was steadfast in giving her son every opportunity to recover, no matter the odds. She was certainly not going to give up. Being the attending physician, I committed to caring for her son as long as she wanted.
After eight weeks in the ICU, the nurses called me to tell me he had opened his eyes. Several days later, the nurses åbelieved his eyes were tracking them in the room. A few more days went by and when I placed my hand into his I thought I felt a slight squeeze. The nurses and I saw some progress every day after that. I could soon tell he recognized his mother. The critical day came where we withdrew his ventilator and he was able to breathe on his own. One week later he said his first words. Several more weeks went by and with lots of help he began to take his first steps. He was going to recover and return home. Praise the Lord!
I then realized that I had to find the cause of his sudden cardiac death syndrome or this could happen again. All our testing so far had not come up with any answers. I hypothesized that he might have a coronary anomaly: This is where the arteries that supply blood flow to the heart are not hooked up in the normal fashion. His mother reluctantly gave me permission to do a heart catheterization. Being an adult cardiologist, I rarely have done a heart catheterization on a teenager and I have to admit, I was a little nervous. I began the procedure in normal fashion, entering the right femoral artery and carefully threading my catheter up the aorta to the heart. My normal routine was to study the left coronary artery first. Usually this takes one or two minutes to find and engage the artery.
After one hour and trying multiple catheters my frustration grew. I could not find it. I decided to move on to the right coronary artery angiogram. I easily engaged the artery and carefully injected dye as our video equipment documented the findings. The mystery was solved: As the dye traveled down the right coronary artery there were collaterals (small feeder vessels that the heart can grow) that filled the left coronary artery. I watched with amazement as the dye slowly filled the left coronary and then began to fill the pulmonary artery where it was hooked up.
This is a very rare coronary anomaly. The coronary arteries should originate off the aorta just above the heart and therefore carry oxygenated blood into the heart muscle. This young man had his left coronary artery originate from the pulmonary artery (which would then carry venous or deoxygenated blood down the artery to the heart muscle). This explained why he had a fatal arrhythmia while playing with his friends. This is somewhat analogous to having a severe blockage in a coronary artery which leads to a heart attack in an adult.
His mother anxiously awaited my arrival to the conference room to hear the results of the procedure. The good news was that we now knew why he had a cardiac arrest and we could fix the problem. The bad news was that he would need open-heart surgery to correct this congenital anomaly.
About two weeks after successful surgery, the young man left the hospital to return home. With intensive physical and cognitive therapy he made dramatic progress over the following year. His mother had been at his bedside daily and prayed to God with great determination and purpose. She understood the meaning of Matthew 7:7-8: “Ask and you will receive. Seek, and you will find. Knock, and it will be opened to you. For the one who asks receives. The one who seeks, finds. The one who knocks, enters.”
Postscript: There may be some readers who noticed that I referenced the pulmonary artery and said that it carries deoxygenated blood. This is indeed true. It is the only blood vessel in the body designated an artery when it really is a vein. The pulmonary artery carries deoxygenated blood to the lungs to become oxygenated, only then to return to the heart.
I must also inform the readers that there were a few purposeful changes made to this young man’s story for confidentiality purposes. Permission was granted to share his remarkable story. In my next column, I will discuss the other more common causes of sudden cardiac death in the young, especially in athletes.
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