My second son is 16 months old. He is a very happy and opinionated baby. To look at him you would never guess that he had heart surgery at 6 days old, a surgery he would not have survived without. His heart is still not "fixed" and it is the doctors' best guess that he will need further heart surgeries "sooner or later".

Prenatal Ultrasounds and the Big "uh oh"

We found out about his heart defect when I was 20 weeks pregnant. At first the ultrasound tech said she "couldn't see the heart properly" and we should come back again. That time she wasn't sure about what she saw so we had to see another tech for an "echocardiogram". That doctor said that our baby had a serious heart defect but he wasn't sure what it was BUT a heart transplant might be a good option. Huh? Like, just get a new heart for a newborn? I knew that wasn't good news.

A few traumatic weeks later we met a pediatric cardiologist who was equally ambiguous about the problem (heart defects are hard to diagnose acurately in-utero due to the differences between the fetal and infant blood flow after that ductus arteriosis closes.) We had to live with teh "not sure what it is" until a few days after our baby was born and only then was a surgical plan worked out. It was also ambiguous and we waited outside the OR to hear what "repair" the surgeon had attempted. That was 15 months ago and our baby is doing great.

The experience of having a child with a serious heart defect has opened my eyes to the wider community of families who share this experience. Congenital heart defects (CHDs) are one of the most common birth defects and it is only in the past 10 or 20 years that more children are surviving into adolescence as the techniques of pediatric cardiology advance. Still, having a child with a 'repaired' CHD is nerve-wracking because there is little or no medical evidence about how these repairs will hold up into adulthood and what kind of life expectancy children with repaired hearts will enjoy.

The Diagnosis 

My son's heart defects is called "Shone's Syndrome, Shone's Complex or Shone's Anomaly". It is a syndrome, which means it is a collection of symptoms and presentations rather than a disease with a known cause. They do not know what causes Shone's Syndrome but can describe it. In Shone's Syndrome the heart has a number of obstructions in the left side, the side that pumps oxygenated blood to the body. To be "Shone's" these obstructions have to be inflow obstructions (mitral valve) AND outflow obstruction (aortic valve stenosis, arch hypoplasia, coarctation or subaortic stenosis). Wren is status 15 months post aortic arch repair and coarcation repair. He has also had a balloon cath of his stenotic aortic valve. He has an abnormal mitral valve which is doing ok right now and SAS (subaortic stenosis). Still, he is doing very well with all this and shows little sign of his heart problems.

The Prognosis

No-one knows if he will continue to be stable indefinitely or need more heart surgery. We are hoping for stability or even improvement as he grows older. The older he is before he needs any valve surgery the better as replacement valves do not grow with the body and need to be operated on again every decade. However, we have heart that in Japan they are already growing artificial valves from bone marrow cells and that these replacement valves grow with the heart and do not require anti-rejection drugs like transplants.We are counting on the ongoing improvements in the cardiology and pediatric cardiology and hoping to share our experiences with other families. It is a frightening time when you receive a pre or post-natal diagnosis of a baby with a CHD but there is so much hope and great things are possible.