The Secret Origin of the Funky Heart!

I’ll be in Washington DC for the Adult Congenital Heart Association‘s (ACHA) Lobby Day on February 10, 2009. The 10th is fine, but in a way I wish it was scheduled for a week later. February 17th, you see, will be the 42nd anniversary of my first heart operation.

I was born in the fall of 1966 and it wasn’t long before my parents realized that there was something wrong with me. My Pediatrician picked up on it fairly quickly, realizing that I had a heart problem, but he wasn’t sure exactly what the problem was. After seeing several doctors who were also stumped, my folks  took me to the Medical University of South Carolina (MUSC).

The doctors at MUSC figured out the problem – I had been born with a Congenital Heart Defect known as  Tricuspid Atresia. In Tricuspid Atresia, the Tricuspid Valve, located inside the heart between the Right Atrium and the Right Ventricle, never formed. But instead of there just being a hole with a missing valve, I have a wall there. Blood flow is completely blocked.

Blood normally flows into the Right Atrium and down into the Right Ventricle; then is pumped out to the lungs. It return to the heart through the Left Atrium, then to the Left Ventricle, and is pumped out to the body. Block the blood flow through the heart and you kill the owner.

What keeps me alive is the fact that there are holes in the wall that seperates the left and right sides of my heart. My blood literally takes the senic route to get through my heart, and on its journey oxygenated blood and deoxygenated blood are allowed to mix. That normally doesn’t happen, and because of it I am Cyanotic – the amount of oxygen in my blood is lower than normal.  I gasp for breath and tire easily;  and my fingernails and lips have a slight bluish tinge. The blueness really becomes pronounced in cold weather.

The doctors at MUSC told my parents that I had Tricuspid Atresia, and that they should take me home and love me, for I was not long for this world. That didn’t sit well with my parents, who went back to my local Pediatrician and said “We need to try something,” even though they had no idea what “something” was. The Pediatrician gave them four places that could attempt a surgical repair: Houston, Texas; Mayo Clinic in Rochester, Minnesota; the University of Virgina; or Johns Hopkins Hospital in Baltimore, Maryland.

Houston and Mayo were eliminated because of the travel distance. The doc threw out Virgina, the medical school he had graduated from, because he felt they didn’t have enough experience with the operation. That really left one choice, and the doc said that he’d get the wheels in motion with the doctors at Hopkins.

That night all hell broke loose. I went into heart failure, with a pulse of 200 and a temperature of 103. It took them five days, but they were able to get things under control at my local community hospital. Meanwhile the doctors at Hopkins were taking the information from my local doctor over the phone and telling him they wanted to see me as soon as someone could get me there.

The folks hit the road, driving into one of the worst snowstorms to hit the East Coast in memory. Things were OK for a while, they finally stopped somewhere in Virgina to rest (this was long before Interstate 95 was completed). During the night, my mother got up to use the bathroom, glanced out the window… and saw it snowing. She woke up my father; we were in the car and moving within 15 minutes.

Every few miles Daddy would pull off the road long enough to wipe off the windshield with an old rag he had found in the trunk. The snow was getting deeper and more and more cars were stalled along the highway, and at one point my father thought about putting Momma and me on a train for Baltimore and catching up to us later. It never came to that, thankfully.

The Hopkins Admissions Office expected three families from out of town to arrive that day, we were the only ones who made it. The doctors at Johns Hopkins examined me not long after I arrived and Cardiologist Richard Rowe gave my parents the news. “He’s down to hours. We have to operate tonight.”

Dr Alex Haller, the Pediatric Cardiac Surgeon, should have done my operation. He was in Washington DC, trapped there because of the snow. Dr. Vincent Gott, Chief of Surgery, stepped in for him. The surgery was known as the Glenn Shunt – a variation of the operation, known as the Bidirectional Glenn Shunt, is performed every day in operating rooms around the world. But at that time it was new, so new that I was the 5th person to survive the operation.

And sometime over the next few days, my father picked up a copy of The Baltimore Sun and gasped. The snow had been so heavy, it reported, that train service into Baltimore had been halted. If he had put us on that train, I would have died.

When I went to Baltimore for the ACHA a few months ago, I had hoped that the schedule would allow me to go back to Hopkins and look around. Not that I expected to know anyone – doctors retire or move on, especially after 42 years. In fact, I don’t remember a single person. I vaguely remember a statue. I’d like to see Him again.

Ten and a half feet tall, standing on a podium, a statue of Christ greets visitors to Johns Hopkins Hospital. His name is Christus Consolator, or The Divine Healer.

I think we’d have a lot to talk about.

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7 Responses to “The Secret Origin of the Funky Heart!”

  1. Katie Says:

    I love reading this story…your heart journey is amazing and I bet it feels great to be one of the first survivors of a serious congenital defect! Your parents did an amazing thing that night and with His will, everything turned out great. Now you’re able to live out His plan for you and touch many lives with your research, ideas, stories and educating us all on the world of living with a CHD. Great for us parents who are raising a child with a CHD. Thank you Steve!

  2. Kim Says:

    I love your presentation of your heart story – very moving and well told. As a heart mom, I could just feel the anxiety of that long drive, the desire to do anything for your child.

    I look forward to the day when my daughter is able to look back and tell her story, maybe not as eloquently, but tell it nonetheless, with many years behind her and that first operation.

    Thanks for sharing!!

  3. carolyn compton Says:

    i have printed this page off to share with my stepson…so that he understands the context of his brother’s heart problems in history. There is nothing like a rip-roaring adventure to excite a 14 year old!

  4. Laurel Nelson Says:

    This is amazing. My own daughter was thought to have Tricuspid Atresia in utero, but after birth, they diagnosed her with a very severe Ebstein’s Anomaly – they told me her Tricuspid Valve might as well not be there since it was so malformed. She also has Pulmonary Atresia. I am always interested in the stories of people who have survived similar conditions, and I am amazed at your outcome – imagine to be the 5th person to survive the Glenn. I know all too often back then anxious parents handed off their children to surgeons never knowing if they would get them back, and most often they didn’t. Did you ever have a Fontan after they started doing those or was it not needed in your case?

  5. Jodi Says:

    What a wonderful stroy! My 3 year old son Cameron has Tricuspid Atresia and Pulmonary Atresia. Since we know so little about future concerns, it is wonderful to hear that there is hope for a bright and long future! Thank you for sharing your heart journey. It gives other heart families hope. Your parents as well as yourself are simply amazing!

  6. Bob Says:

    Just an incredible story, extremely well written but please don’t be in a hurry to get to see Him in person – not when you are so young and He did so much to keep you alive – He must have a special purpose in mind for you.

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