Huntington's Disease Can Affect the Heart

Huntington's Disease (HD) is an autosomal dominant progressive neurodegenerative disorder. In addition to the central nervous system disturbance the ailment causes, there is increasing evidence the genetic abnormality also affects peripheral organs and tissues.

Huntington’s Disease (HD) is an autosomal dominant progressive neurodegenerative disorder. In addition to the central nervous system disturbance the ailment causes, there is increasing evidence the genetic abnormality also affects peripheral organs and tissues.

In a study presented April 22 during a poster session at the 2015 annual meeting of the American Academy of Neurology, Christopher Stephen, MD, a neurologist at Massachusetts General Hospital in Boston, MA, and colleagues reported on electrocardiogram results in a sample of patients with Huntington’s Disease.

They found that 25.3 % of the 598 early HD patients, all over age 18, had abnormal electrocardiograms (ECGs).

Nearly all of the patients were in the first 2 stages of Huntington’s Disease.

After controlling for medications with cardiac effects and other relevant medical conditions, the team found 23.7% had marked bradycardia. Prolonged intraventricular condition was also significantly higher in these patients (4.3% in HD patients vs. 1.3% in non-HD populations.)

“The increased prevalence in conduction abnormalities and bradycardia in early Huntington’s Diseasesuggest possible compromise of the cardiac bundles and sinoatrial mode, which could lower the threshold for arrhythmia and aggravate cardiac failure,” Stephen concluded.

The results are important in part because they suggest physicians and researchers should use caution in prescribing medications or experimental therapies that may have cardiac toxicity.