Jean Parish kindly offered to share her story with others who visit Left Heart Matters. It is the story of Rachel Elizabeth

On May 29, 1997, my husband Scott accompanied me to a routine sonogram test. I was 22 weeks pregnant and everything was going well. As the test concluded, the technician stepped out to consult with the radiologist. This all seemed routine until the radiologist came into the room. She stared grimly at the picture of our baby's heart on the screen. She told us there was a heart abnormality and she would need to refer us to a specialist to make a diagnosis.

Of course my husband and I were shocked and upset. The next day, we went to an OB specializing in high-risk pregnancies. After a lengthy sonogram, she diagnosed our baby's condition as HLHS. She explained the condition in detail to us. The doctor said the cause was unknown, but reassured us that nothing I ate, drank, or did caused this to happen. We cried as she spoke, but we continued to listen to her carefully.

The doctor explained our options to us:

1) Terminate the pregnancy
2) Carry the baby to term and then take her home to die (referred to as compassionate care)
3) Try to get a heart transplant
4) Attempt a three stage surgical procedure, startng with the Norwood, to repair her heart.

Chances are slim to none to obtain an infant heart for transplant in Texas. It is likely that our baby would die waiting. Then this "Specialist" started to explain the Norwood procedure. We found out later her information was extremely outdated. This doctor told us that the Norwood was experimental and the Houston medical community deemed it unethical to perform because the success rate was so low. This doctor also told us that HLHS is often seen in babies with defects that are "incompatable with life." She continued with the sonogram to look for further defects as she asked us how we felt about terminating the pregnancy. Texas law would prohibit termination after week 24, so we had less than 2 weeks to decide our unborn daughter's fate.

Unaware we had the wrong information, we went home devastated. We decided to take the advice of the doctor and schedule an amniocentesis. This test would tell us if our baby girl had further birth defects. However, the results would not be back before the 24 week deadline. Although I did not want to terminate the pregnancy, I could not imagine carrying a baby full term, knowing she would die soon after birth. We were going to have to schedule what we called "option #1" before we found out the results of the amnio.

Meanwhile, we looked up information on the Internet about HLHS. We were surprised to find a wealth of knowledge. Web pages such as this one and sites authored by parents inspired us to furthur consider the 3 stage procedure. We met with a cardiologist, Dr Kovalchin, at Texas Children's Hospital here in Houston who set us straight about the surgical option. He told us about Dr Fraser, a well respected cardio-thoracic surgeon at TX Children's and his success with the palliative surgeries. We talked via phone and email to parents of children with HLHS. Dr Fraser, who was out of town at the time, phoned us to discuss the surgeries and his experience with HLHS babies. By the time we got the results of the amnio, we had already decided to go for the surgical option. However, I jumped for joy when I found out that the test did not indicate there were further defects. My OB, Dr Brooks, referred me to a different specialist to consult, but she remained optimistic about the situation. I continued to see the cardiologist for fetal echocardiagrams. My OB and I planned an induced labor in the hospital adjoining TX Children's. A neonatologist and her team would be present and ready. Dr Brooks delivered my baby girl, Rachel Elizabeth, on September 30, 1997. It was a vaginal delivery without complications. She was 8 lbs, 20 inches long. Rachel scored 8 and 9 on her Apgars. I held her briefly before Scott escorted her to the NICU. Her challenges were just beginning, but she was off to the best start we could hope for.

Please feel free to email me. I would be glad to tell you more about our experience. I wanted to write about our prenatal diagnosis to help couples in the same situation. It is so important to have doctors you can trust and a support system of family and friends. I cannot say enough good things about Dr Fraser and TX Children's Hospital. Rachel continues to receive excellent care from the cardiology staff.

As I am writing this, my beautiful baby girl is in her swing next to me, cooing and looking around at everything. She celebrated her 6 week birthday this week. I realize we were blessed to find out about Rachel's heart months before she was born.

Jean Parrish
Houston, TX
jean@entech.com