Decreasing deaths in infants with hypoplastic left heart syndrome (HLHS): Our treatments are working

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HLHS was once a uniformly fatal diagnosis. HLHS is also one of the most common and most severe forms of single ventricle congenital heart disease.

In the last 20 years, the options for treatment of HLHS have changed dramatically with heart transplantation and the development of the staged Norwood procedure which results in a Fontan physiology with the single right ventricle as the systemic ventricle.

In this issue of The Journal, Gordon et al report on the mortality for infants with HLHS in California from 1990-2004. As shown in the Figure, annual mortality is cut in half over the study period. Although this is a major success, it is also important to note that there is still a substantial number of deaths attributable to this congenital heart defect. This underscores the fact that although treatments have improved, they are still far from universally successful. Improving approaches to care of these infants are still needed.

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