Interviewer: Dr. Naidu, thank you for taking the time to do this interview today on
hypertrophic cardiomyopathy and the current and novel treatments for the disease.
Could you start with an outline of what hypertrophic cardiomyopathy is?
Prof. Naidu: Excellent. Yes, I'm happy to be here again.
Hypertrophic cardiomyopathy is a cardiac genetic disease
which is actually fairly frequent.
We thought this was about one in 500 people.
It turns out, it's probably one in 200 or one in 300 with expression of
the disease at various time points throughout someone's life who has inherited a gene.
It results in a hypertrophy or
a thickening of the heart muscle which can be any location of the heart,
but is most typically in the basal septum.
This usually happens in the teenage years or in the 20s and 30s,
although people can be diagnosed late in life.
Oftentimes, even if the disease has been quiescent from a hypertrophy standpoint,
the culmination of the hypertrophy and obstruction or
other sequela of the disease may result in
heart failure symptoms or other symptoms later in life.
This makes it very challenging because you would think of
most genetic diseases as presenting early in life, yet,
we know that there's a disconnect here and that
some people may have significant hypertrophy and
significant hypertrophic cardiomyopathy at an early age and yet be very functional.
Interestingly, many of the athletes,
roughly 40-50 percent of athletes who die suddenly of cardiac arrest end up having
hypertrophic cardiomyopathy at autopsy or when evaluated if they survived that episode.
Just taking a step back, it results in hypertrophy,
and that may be clinically unseen for many years.
Depending on the location of hypertrophy,
it may cause a number of symptoms.
If there's a lot of hypertrophy,
typically over two-and-a-half to three centimeters,