More children surviving dilated cardiomyopathy without heart transplant

Dilated cardiomyopathy occurs when the heart is enlarged (dilated) and the pumping chambers contract poorly (usually left side is worse than right). It can have genetic and infectious/environmental causes.

Researchers analyzed the clinical outcomes of children with dilated cardiomyopathy in the NHLBI Pediatric Cardiomyopathy Registry (PCMR) and divided them into two groups based upon year of diagnosis: Era One (1990-99) included 1,199 children; Era Two (2000-09) had 754 children. The median age at diagnosis was 1.6 years in the first group and 1.7 years in the second.

Researchers found:

• Fifteen percent of patients (291) from both groups died without receiving a heart transplant.
• Era Two patients were more likely to be treated with heart failure medications: ACE inhibitors (71 percent vs. 62 percent); beta blockers (24 percent vs. 6 percent); and diuretics (89 percent vs. 84 percent).
• Era One patients were 1.5 times more likely to die than Era Two patients.
• Heart transplantation rates were not significantly different between the two periods.

"Children with dilated cardiomyopathy have better survival in the more recent era, which appears to be associated with factors other than availability of transplantation as that was equally prevalent in both eras," said Rakesh K. Singh, M.D., M.S. the study's lead author.

Dilated cardiomyopathy accounts for about 55 percent of all childhood cardiomyopathies. It's detected in about one per 175,000 children each year in the United States, according to the PCMR Database.