Future good for young people with hypertrophic cardiomyopathy

15th December 2015

Young people with hypertrophic cardiomyopathy receiving up-to-date care have good outcomes, says a new study.

Historically, young patients with the disease have been thought to be at greater risk of complications, including dying suddenly.

But a new study from America of young people aged between seven and 29 shows that low mortality can be achieved with contemporary management strategies.

Trial authors, including Dr Barry Maron, said success was based on risk stratification and consensus guidelines.

This included looking at major risk factors including family history of sudden death, unexplained recent fainting, thickness of the left ventricular wall, ventricular tachycardia, blood pressure response to exercise.

He said ICD therapy was life-saving in a significant number of patients, but there was a significant problem with inappropriate shocks.

The researchers looked at 494 young people with hypertrophic cardiomyopathy for between two and 12 years. During the course of the study 95 per cent of patients survived. Eighteen patients died, some suddenly but others from progressive heart failure and heart transplant complications.

When including only HCM-related deaths, five and ten year survival were 97 per cent and 94 per cent, respectively.

These outcomes were similar to those previously reported in middle-aged HCM patients. Approximately half (231) of the young people had had internal defibrillators of which 212 were implanted for those thought to be at risk and 19 for those who had already had an event . Inappropriate shocks occurred in 18 per cent of those with an ICD.

Reference: Hypertrophic Cardiomyopathy in Children, Adolescents and Young Adults Associated With Low Cardiovascular Mortality With Contemporary Management Strategies. Maron BJ, Rowin EJ, Casey SA, et al.