Think of a container that shrinks and still has to send out quantities of liquid. Over time, its business tends to become less efficient. Something similar happens to the heart in those suffering from hypertrophic cardiomyopathy (according to the international definition HCM), a disease that can be hereditary and can arise at any age.
At what age does it manifest itself
In this situation, which is recognized above all between the ages of 40 and 50, a chronic progressive condition occurs: the excessive contraction of the heart muscle and the reduced filling capacity of the left ventricle can make it difficult for blood to circulate in the rest of the body. resulting in the development of debilitating symptoms and cardiac dysfunction.
The most frequent form
Not all of these forms are obviously the same. In the most frequent case, the so-called obstructive form, the outflow tract of the left ventricle, where the blood leaves the heart, is blocked by the thickened heart muscle. This carries an increased risk of atrial fibrillation, heart attack, heart failure and sudden cardiac death, although rare. The most frequent cause in these cases is the mutation of the sarcomere proteins that make up the heart muscle.
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For cardiology, this condition represents a major challenge. And research is working to find new solutions, remembering that the situation must be recognized in time and specific ways of treatment must be found. However, a few pieces are added to the mosaic of knowledge also in terms of treatment, thanks to the use of innovative drugs.
Studies on innovative drugs
Proof of this is what emerges from the Phase III study VALOR-HCM. Research has shown that the addition of mavacamten, a first-in-class cardiac myosin inhibitor, significantly reduces the need for septal reduction procedures (in technical term SRT) in patients with severe symptomatic hypertrophic obstructive cardiomyopathy who are candidates for SRT. at baseline, according to the American College of Cardiology / American Heart Association (ACC / AHA) 2011 Guidelines. Study participants were on treatment regimens at maximum tolerated levels when they entered the study and remained so for the duration of the study.
The good news emerges from the congress ofAmerican College of Cardiology. At 16 weeks the study objectives were met. Of the patients treated with mavacamten, 82% no longer needed SRT and no longer met the criteria for SRT according to the 2011 ACC / AHA Guidelines compared with 23% of patients who received placebo.
Patients in the mavacamten arm also demonstrated, with a high level of statistical significance compared to the placebo arm, a reduction in left ventricular outflow tract gradients, an improvement in the NYHA class (classification that distinguishes heart failure), an improvement in measures of quality of life and an improvement of cardiac biomarkers. All without any particular needs in terms of safety having been identified.
“VALOR-HCM is based on the results of the Phase III EXPLORER-HCM study and shows that mavacamten is an effective option for patients with severe symptomatic obstructive HCM who meet the criteria for SRT, according to the guidelines.” Milind Villagei, MD, MBA, director of the HCM center e direttore del clinical operations, Heart, Vascular & Thoracic Institute, Cleveland Clinic. “The data presented today are clinically significant and have demonstrated the potential to influence the parameters that make patients eligible for SRT.”
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