HLHS, or Hypoplastic left heart syndrome, is a common form of congenital heart disease. The conditions present with an underdeveloped left ventricle that is unable to pump blood efficiently through the heart. In many cases, the condition is diagnosed via ultrasound giving parents the ability to choose heart surgery immediately after birth.

In a study completed by researchers from the Children’s Hospital of Philadelphia, 240 fetuses were diagnosed with HLHS. Of these cases, 68% fell into the standard risk category and 32% in the high-risk category. Infant heart surgery, stage one called the Norwood procedure, was completed on 185 HLHS patients. Of that 185, 155 lived through the procedure and 30 died during or after surgery. The standard risk patients were more likely to survive. About 93% of the standard group, which makes up about 2/3 of all HLHS cases, survived the first stage of heart surgery.

Researchers believe more infants diagnosed with standard HLHS may benefit from the Norwood procedure and subsequent operations. Standard risk makes up 2/3 of all HLHS cases. The purpose of the study was more than just proving a surgery can be effective. Children born with this condition can live long, prosperous lives if proper treatment is given. Researchers believe that proper treatment may be heart surgery.


Source: Jack Rychik, M.D; Anita Szwast, M.D.; Shoba Natarajan, M.D.; Michael Quartermain, M.D.; Denise Donaghue, R.N. M.S.N.; Jill Combs, R.N., M.S.N.; J. William Gaynor, M.D.; Peter Gruber, M.D.; Thomas Spray, M.D.; Michael Bebbington, M.D.; and Mark Johnson, M.D.; all of The Children's Hospital of Philadelphia. Ultrasound in Obstetrics and Gynecology.27 October 2010.