Caylen with Heterotaxy and several other major and minor defects

Caylen with Heterotaxy, Dextrocardia, Atrial Ventricular Discordance, Sick Sinus Node syndrome, Double Right Outlet, Transposition of the Great Arteries (TGA), Ventricular Septal Defect (VSD) and Pulmonary Stenosis, as well as several other minor defects

A mother’s story of how she found out and what she went through.

Our daughter, Caylen, was born with CHD. She has heterotaxy (her organs are transposed), dextrocardia, atrial ventricular discordance, sick sinus node syndrome, double right outlet, Transposition of the Great Arteries (TGA), Ventricular Septal Defect (VSD) and Pulmonary Stenosis, as well as several other minor defects. We had no idea that Caylen had CHD while I was pregnant. I had multiple ultrasounds and a fetal echo. No one saw anything. When I was 35 weeks pregnant, my amniotic fluid kept increasing. The doctors thought I might be developing pre-eclampsia, yet my blood pressure was low. I gained 30 pounds of fluid my last few weeks. At 38 weeks, it was decided that they would take Caylen by cesarean section. When Caylen was born, she wasn’t breathing, it was more like a hiccup. The doctors immediately sent her to the NICU. They came to us hours later, telling us she probably wouldn’t live through the night. However, our little girl is one heck of a fighter! They still hadn’t figured out what was wrong with her. Talk about completely baffled! Caylen’s major CHD, Atrial Ventricular (AV) discordance is among the rarest of CHD’s. It took several days for them to make sense of her heart’s anatomy and to figure out what was wrong. At five days old, they corrected her mal-rotated intestines. She kept overcoming all the odds they placed on her.

Caylen’s VSD, along with the hole in her heart that we all are born with, saved her life, allowing her blood to mix. She was able to go home at a month old. However, with her sats in the 50′s and 60′s, we didn’t know if she would live to see a year old. Caylen would need a procedure that hadn’t been used in 40 years, according to her doctors. They planned to operate when she turned one. Unfortunately, the pressure in Caylen’s lungs started climbing steadily. She had been protected from pulmonary vascular disease by the pulmonary stenosis. If she developed that, our only option would be a heart-lung transplant. So, at four months old, her doctors began looking for a surgeon that still knew how to perform the operation she needed. Fortunately for us, Dr. Edward Bove at the University of Michigan’s Pediatric Congenital Heart Center had modified the Senning (or Mustard) procedure for use on Hypoplastic Left Heart Syndrome (HLHS) / Hypoplastic Right Heart Syndrome (HRHS) cases. He could use this to help rebuild Caylen’s heart.

Off we went to Michigan from North Carolina when Caylen was five and a half months old. We had to drive, as it was too dangerous to fly. We had spent the previous six weeks praying so hard for her and for Dr. Bove. We were absolutely terrified that Caylen wouldn’t survive the surgery. I don’t think I have ever cried so much in my entire life from the time she was born until after the surgery. All I could do was hold her and cry. Our faith had always been strong and a major part of our life. It was shaken at this point, but it was all we had to hold on to. Since Caylen’s heart was so strange a landscape, it took a lot to map it for the surgery. Her atria were correct, the ventricles and the greater arteries were correctly aligned but backwards. Dr. Bove had to rebuild Caylen’s atria, using donor material to create baffles to redirect blood flow. She had arteries widened and added, the VSD closed and the double right outlet corrected. It was a long surgery on Dec. 12, 2007.

After surgery, Caylen was vented for two weeks. She was on over 20 meds, too. Her heart kept wanting to beat slowly, so they tried speeding it up. Then she started having SVT (super ventricular tachycardia sp?) where her heart rate was over 270 bpm. They tried several drugs to stop them, finally going to amioderone, a very toxic drug. Finally, after much discussion and arguing, another heart surgery was performed to implant a pacemaker on Jan. 2, 2008. Nearly two weeks later, after nearly six weeks in the hospital, she was finally released to go home to NC. Caylen had problems with arrhythmia when we got home, especially after the amioderone wore off. Her meds were adjusted several times, until the episodes settled down. She was deemed stable enough for us to move to Mississippi to be near my family.

Unfortunately, Caylen’s arrhythmia became problematic again in Nov. of 2008. She required a cardioversion to stop it. Then, again the following April, she had to have another one. At that point, it was decided she needs a laser catheter ablation. The problem is that her heart is too small for it to be efficient at that point, and that Caylen doesn’t have an artery that goes directly to her heart, which makes the procedure very risky. She was put back on amioderone, in additon to lasix and flecainide to control the arrhythmia. Our hope is that she will be able to stay on it until she is four or five, when the efficiency rates for the ablation will go much higher.

To look at Caylen, you would never know something is wrong with her, unless you see the railroad tracks of scars on her torso. She is full of energy and life, hardly ever stopping or slowing down unless her heart is off rhythm. She is a testament to God’s will, love and grace and His never ending supply of miracles. We have no idea how long Caylen will live or if she will have to have more heart surgeries in the future. She is a very rare case. At this point, she is considered to be doing quite well. The changes to her heart are growing with the heart at this time. As to whether they will continue to grow well as her heart changes will determine her outcome.

Caylen’s CHD has led me on a journey that I never thought I would take. No one mentioned CHD to us when I was pregnant. I was a high risk pregnancy to begin with: I was forty years old, overweight, diabetic and on several medications. We were told my chances of getting pregnant anyway were less than 3%. I was on birth control. Obviously, God had other plans. Some good has come out of this - my daughter is alive, the doctors are learning more about her defect, and I have been led to start a Mended Little Hearts chapter here in Mississippi, where there are no support groups for CHD families. God has given us so much, I wanted to give something back. In doing this, we are helping families and raising awareness of CHD.

We are so blessed with Caylen. She’s gone through so much, but there are CHD children out there who are going through so much more, whose prognosis’ are much worse than hers. We remind ourselves of that everyday. We also tell everyone we come in contact with about Caylen and CHD. Not because we want Caylen to be a victim, which she is NOT, but to show people what God can do and to put a face on CHD to those who haven’t seen it before. It’s become our life mission, for my husband and myself, to spread awareness of CHD and to advocate for more research and money for pediatric CHD. If our daughter can fight so hard to live, we can fight just as hard for her and for children like her.

Wrote by Erin and David Ayscue
Clinton, Mississippi
February 2010

To view Mended Little Hearts of Mississippi, click here
or join their Facebook group, click here
Mended Little Hearts provides hope and support to children, families and caregivers impacted by congenital heart defects in order to extend and improve quality of life.

Erin Ayscue has kindly given CHD-UK permission to use the photographs on this page.