IMH Heart Connections

Story told by Katherine's mother, Marty Evans.

Katherine "Katie" Evans was born on April 7, 2006. She was my third child so she came quickly and appeared completely healthy at birth. About 5 hours after she was born while I was resting in my room, a neonatologist came in and told me that Katie would have to be transferred to the Newborn Intensive Care Unit (NICU) because she was having "dusky" spells. She spent a day and a half in there undergoing various tests as they were trying to determine what was causing the dusky spells. Finally, about 36 hours after she was born they decided to do an echocardiogram. At the "echo" they determined that she was a Hypoplastic Left Heart Syndrome (HLHS) variant. She had a critical coarctation (very narrow) of the aorta with a hypoplastic (small) aortic arch, left ventricle, mitral valve and aortic valve hypoplasia, a biscuspid aortic valve, tricuspid valve regurgitation, and an atrial septal defect. She was considered a variant because she had a left ventricle (some HLHS babies don't have a left ventricle at all). She was immediately transferred to the children's hospital here in town and was given a prostaglandin IV to keep her alive. The physicians knew that she would need surgery to save her life, but they debated which surgery to give her. They concluded that at that time her left ventricle and the surrounding structures were too small to support normal, biventricular circulation. It was decided that Katie would undergo the Norwood. This is a highly complex surgery where they begin to covert the heart to single ventricle circulation. Katie had her first surgery at 1 week old--on Good Friday. She was very sick afterwards and had a complicated and drawn out recovery. We were able to take her home when she was 5 weeks old.

That summer Katie had a hard time. She was not able to drink from a bottle. She would sweat after one ounce of her bottle so she had to be on an NG tube (the yellow tube that goes from their nose to their stomach). I remember all summer as I held her noticing that she smelled like her medicine because she would sweat all of the time. Her heart function was just horrible. When I held her little hands I noticed that our hands were such different colors because of her lack of oxygen. Her sats (oxygen saturation) were so low that she was literally grey. She had 2 catheterizations that summer where they ballooned her aorta. We couldn't take her around anybody or let anybody hold her. That was a long hard summer.

By fall, it was time for her second surgery. We traveled to Texas Children's Hospital in Houston for this surgery. It was a long trip for our family and it was hard to be away from my other two kids, but little did we know how much it would change her entire life.

After her pre-op cath, the cardiologist came out and totally surprised us. He told us that her left ventricle was the largest left ventricle he's seen of somebody considered HLHS. He told us that he thought she could survive with a normal biventricular circulation. We thought he was crazy. The next day the surgeon, Dr. Charles Fraser, came to Katie's room and talked to us very seriously about Katie's left ventricle. He told us that he thought she had a chance at normal circulation. He told us that he might undo her Norwood and do a complete repair on Katie's heart. He said that he really had to look at Katie's heart (in surgery) and make the final decision to make sure that the valves would really support this radical change. He told us that he could never relegate a child to a single ventricle if there was a possibility of a normal heart. Dr. Fraser said that if it worked out that they did this surgery, Katie's life would change drastically. He said that this surgery would add 65 years on to her life expectancy. We really couldn't believe what we were hearing. It seemed unimaginable. It seemed like a miracle.

We had to wait in the hospital for a week, but the day finally came. We felt nervous but very calm at the same time. Katie went into surgery at 11 am. Dr. Fraser told us that they would call us from the Operating Room and tell us which surgery they were going to do--the Glenn (what we came for) which had a very small mortality rate or the complete repair, which was much more complex and had a 10% mortality rate. We prayed and prayed that Dr. Fraser would see things very clearly and make the right decision. They called us about 3 hours into surgery and told us that Dr. Fraser had decided to do the complete repair. We were elated. We cried and just fell to our knees and thanked God. We couldn't believe that Katie had this chance. It ended up being a very long day, but finally after 10 hours of surgery, we received the call that Katie was off of bypass and was doing great. Within the hour we got to see Katie. She looked wonderful! I remember walking in her room in the ICU and first looked at her and then immediately looked at that monitor to see her sats. They were 100. It was truly a miracle.

About 2 months after her surgery, we were able to take out her NG tube because she was eating all of her food by mouth--without sweating! She started to roll, crawl, walk and now she runs! Katie is almost 2 now and completely keeps up with kids her age. She's off all of her heart meds and goes to the cardiologist only every 6 months now. It is likely that she'll have to have her aortic and mitral valves replaced in the future, but for now everything seems to be adjusting well to her normal, bi-ventricular circulation.

We are so grateful for the journey that Katie has taken us on. It has been hard, but our whole family has been forever changed for the better by this experience. I know that Katie was supposed to be born with her special heart. I know that this was supposed to be her plan and her story.