What is HLHS (Hypoplastic Left Heart Syndrome)

What is CHD?

Congenital heart disease, or CHD, is the most common birth defect.  It occurs in almost 1 out of every 100 live births.  CHD is a spectrum of abnormalities occurring during development in the womb.  CHD ranges from very minor defects requiring little or no invasive treatment to very serious defects or syndromes involving multiple defects or systems.  These defects may require numerous surgeries or transplantation.

What is HLHS?

Hypoplastic Left Heart Syndrome (HLHS) is a rare heart defect.  HLHS occurs in one out of every 5,000 births.  Each year, approximately 1,000 babies are born with HLHS in the United States.

The cause of HLHS is unknown.  In the womb, the left side of the heart does not develop properly, typically growing to just a fraction of the normal size.  The easiest way to think of HLHS is that these children are essentially born with half a heart.  Very often children with HLHS are otherwise healthy, although HLHS may occur in combination with other disorders or multiple defects.

If not treated surgically, HLHS is uniformly fatal in the newborn period.  The surgical repair requires three surgeries performed at birth, at 4-6 months-of-age, and at 18-24 months-of-age.  Dramatic improvements in survival have been made in the past 25 years, from a universally fatal disease to one with current survivals of about 75% at five years.  With repair, these children can be happy and relatively healthy kids, with an excellent quality of life.

However, much work remains to be done.  Research remains the key to not only improving survival, but quality of life.  With continued research, ever-improving methods of treatment and earlier detection are increasing the chances for these children to not only survive, but to enjoy the opportunity to live full active lives with minimal restrictions.