My Story: Straight From The Heart

By Susie Saunders, M.D., Pediatric Alliance — Arcadia Division


Did you know today kicks off Congenital Heart Defect (CHD) Awareness Week? I used to think of February as the month of romance, love, and Valentine’s Day, but now this month signifies something much more meaningful – it reminds me of all the tiny hearts and families affected by CHD. The most common birth defect, CHD affects 1 out of every 120 newborns, ranging in severity from minor to very complex.


Congenital Heart Defect Awareness Week

                  February 7-14, 2014



My story begins with my husband. He has a bicuspid aortic valve, the most common type of CHD, and although present since birth, he remained undiagnosed until his early 20s. He remains without symptoms, as will the majority of individuals with a bicuspid aortic valve, until the 6th or 7th decade of life, and undergoes annual ultrasounds of his heart.

When we became pregnant with our first child, we were told the risk of our child having CHD would be 4%, which is more than 4 times greater than the 0.8% incidence in the general population. Looking back, I remember thinking 4% is nothing…unless you are a part of the 4%, which we now belong.

We were told that our child had a heart defect during our routine 20-week ultrasound. We were informed that the heart defect was in the “gray zone” meaning the significance wouldn’t be truly known until birth. How was that possible given all of the advances in modern medicine?  Her defect could range in severity from coarctation of the aorta (a narrowing of the part of the heart that supplies blood to the lower extremities) to something much more complex: hypoplastic left heart syndrome (the left side of the heart, which pumps blood to the entire body, does not develop). Either way, I knew that it meant I wouldn’t be able to hold my baby after birth. I wouldn’t be able to feed her or change her first diaper. It meant an admission to the cardiac intensive care unit (CICU), and worst of all, she would not survive without open-heart surgery. Devastation doesn’t begin to describe the way I felt.

All the emotions that come with celebrating the happiness and excitement of a first baby were changed to constant feelings of fear and uncertainty for the remainder of the pregnancy. Plans, such as gender reveal pictures and baby showers, changed. Lots of questions ran through my mind. What do we do about daycare?  What do we do about work? How much time will she need to be in the hospital? All of our plans changed in an instant.

Genetic syndromes and chromosomal abnormalities can cause some heart defects, so we were faced with agonizing decisions. Do we do chromosomal and genetic testing? Do we terminate the pregnancy? Do we proceed as initially planned? Do we do nothing?

Lilah was born August 31, 2013. My husband got to hold her briefly and show her to me before she was taken to the neonatal intensive care unit prior to transport to Children’s Hospital of Pittsburgh.  We were presented with multiple different scenarios during her first 48 hours in the CICU. I remember crying happy tears the first night because we were told her heart defect was “just a coarctation of the aorta.” This is the defect we wished for because it was repairable and meant a single open-heart surgery. The following day it quickly turned into our worst nightmare – hypoplastic left heart syndrome. This diagnosis meant three palliative open-heart surgeries and almost certainly a heart transplant in the future.

Lilah underwent her first surgery at one week of life. She came out of the operating room with two poles of medications, chest tubes, multiple intravenous lines, heart pacing wires, and a breathing tube. Having trained at Children’s Hospital as a pediatric resident physician, this was not new to me, but seeing MY own child that way was HORRIBLE. My perspective for families and their unique experiences changed forever. Over the next two weeks, one by one the supporting devices and medications were taken away. The first surgery, called the Norwood procedure, is one that many babies did not survive in the 1980s during the early days of the procedure. Thanks to research and advances in surgical techniques, medications, and postoperative care, this surgery now has close to an 85% survival rate. However, there needs to be more funding to support pediatric research to further improve long-term survival and treatment options for patients with complex CHDs. We were lucky: Lilah survived one of the most difficult and complex surgeries performed on these tiny hearts. We would be taking our baby home, a blessing that many families in our situation don’t get to experience.

The next month was spent at weekly appointments with cardiology and the cardiothoracic surgical team to closely monitor her progress. At home, she was fed every three hours through a feeding tube in her nose. The second surgery (called the Glenn) is usually done around 3 to 4 months of age but during one of our appointments in October, we found out she was in heart failure and required an immediate heart catheterization. This resulted in a second admission to the CICU and undergoing her second heart surgery earlier than expected. On day 7 of hospitalization and 2 months of age, she underwent her second open-heart surgery. Once again, many intravenous lines, medications, and tubes surrounded our tiny baby. Slowly but surely these disappeared again and we were discharged home.

Except for a few minor setbacks, Lilah’s heart story has been one of strength and triumph. I think about her heart and future every day, but often times it’s only when she receives her daily medications, goes to a cardiology appointment, or when I see her scars. I have met families and patients who have their own heart stories, learned from their heart experiences, and shared in their joy and pain. Lilah is now 17 months old and a VERY active, happy toddler.  We are amazed with her every day! She will need her third heart surgery this year, but for now we are taking it one day at a time and appreciating every second we have with our precious baby girl.


***Lilah with her beads of courage — this is a program at Children’s Hospital of Pittsburgh which gives children with complex medical issues beads for all the procedures and testing done to them.***