We can’t do anything.
That is what parents of babies diagnosed with Hypoplastic Left Heart Syndrome (HLHS) were told not many years ago. The word Hypoplastic means “underdeveloped”, and that is exactly what has happened: the heart’s Left Ventricle is much too small to pump blood to the body. The hypoplasia extends much further than the Ventricle; the Aortic and Mitral valves are tiny (or nonexistent), and the Aorta itself is tiny. The child is alive only because of a Patent ductus arteriosus, a “built in” heart defect everyone is born with.
The Ductus Arteriosus is a small connection between the Pulmonary Artery and the Aorta that allows the heart to function while still in the womb. Once the child is born, the connection will close by itself, usually in less than two weeks. A connection that is “stuck open” — a Patent ductus arteriosus — is easily corrected. But in this case, when the ductus closes, blood flow drops and the child goes into shock.
HLHS was the defect the doctors had no answer for. Obviously, a surgical repair was the only option, but how? How do you fix a heart that is, for all intents and purposes, missing the left half? A heart transplant was an option, but rarely was an infant’s heart available, and there was almost no time to find one.
But in the early 1980’s the answer came. Rather than try to fix everything at once, make the repair in stages. Three operations, (known collectively as the Norwood Procedure) performed in sequence, allowed the child to survive. Certainly it was understood that this wasn’t a cure, but the child could live if he or she received lifelong medical care.
Fast forward to 2009. The first generation of HLHS survivors are now in their early to mid 20’s. And with no older survivors, no one has any idea what future health problems may affect them. Like Edmund Hillary climbing Everest, no one’s been here before, and no one has a clue what's ahead.
The CHD Blog and Adventures of a Funky Heart will both be in Washington, DC on February 10, 2009 to support the National Congenital Heart Coalition (NCHC) effort to create a national registry of patients with heart defects. The NCHC is made up of several different CHD organizations, working together for the first time. The American College of Cardiology, the Adult Congenital Heart Association, It's My Heart, the Congenital Heart Information Network, Mended Little Hearts, and the Children's Heart Foundation have all come together to work for this important cause. (The group information is taken from the cover of the informational packet that was mailed to attendees; there may have been more groups that have joined the effort since then!)
If data is gathered about those of us living with a Congenital Heart Defect right now, it could be analyzed and used to predict our future health care needs. It can also be used to make things a little easier for the next generation of CHD patients. As an example, I take 11 different medications. Yes that is a lot, but some CHD’ers take more, and some take less. If the knowledge gained from a national registry could eliminate one medication that each of us has to take, the savings would be phenomenal.
Much too often you read in your local newspaper about the child who needs surgery for a major heart defect (Infant Survives Heart Surgery, reads the title of the one and only article about me in the local Daily Planet) but what happens after that? Usually you don't see a follow up article in the local press. But Cardiac Kids grow up; we get jobs, pay our taxes, fall in love…
... In other words, we live our lives.