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HLHS Information

What is Hypoplastic Left Heart Syndrome (HLHS)?

HLHS is a Congenital Heart Disease where the left side of the heart has failed to develop properly. There are varying degrees of malformation in the Hypoplastic Left Heart.

  • The left ventricle is usually very small or non-existent.
  • The aortic valve may be narrowed or closed and the aorta itself may also be very small.
  • The left atrium is sometimes small and the mitral valve between the left atrium and left ventricle can also be narrowed or closed.

These defects put extra work on the right side of the heart, so chambers and blood vessels on this side may be enlarged or stretched larger than normal.

HLHS is considered to be one of the more severe CHDs. Although there is no cure for HLHS, advances in paediatric cardiology and surgeries mean that more children are being given a chance at life.

Approximately 80% of the babies that undergo the palliative surgeries survive, however the long term outlook for these children is unknown. Treatment of HLHS was pioneered in the early 1980's so there are quite a few adult HLHS survivors.

INTERNATIONAL CLASSIFICATION OF DISEASES (ICD-9)
746.7: Hypoplastic left heart syndrome - Atresia, or marked hypoplasia, of aortic orifice or valve, with hypoplasia of ascending aorta and defective development of left ventricle with mitral valve atresia.

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