di Francesca Conti
First Course on the Treatment of Obstructive Hypertrophic Cardiomyopathy in Florence
The first edition of the event will be held on June 18th at the Meyer Health Campus in Florence Course on the treatment of Obstructive Hypertrophic Cardiomyopathy: focus on cardiac surgery and medical therapy
The course is designed to present the impact of obstruction on the quality of life and prognosis of patients with hypertrophic obstructive cardiomyopathy (CMIO). The cardiac surgical experiences gained over the last twenty years in Florence, at the AOU Careggi under the guidance of Prof. Pierluigi Stefàno, and at the Monza Polyclinic with Dr. Paolo Ferrazzi will be illustrated.
During the course, the methods of patient selection and individualized planning of the type of intervention for the different forms of CMIO will be presented.
The experiences gained so far with "classic" drugs, such as beta-blockers, and in particular the most powerful, Nadolol, and Disopyramide, will also be discussed, as well as the results of recent clinical trials conducted with a new class of drugs, the cardiac myosin, which include Mavacamten, already approved by AIFA, and Aficamten, which awaits approval from the US, European and finally Italian drug agencies. Drugs capable of attenuating the functional, energetic and clinical alterations associated with CMIO, capable of effectively and safely reducing outflow obstruction and, in some cases, postponing or avoiding surgery.
Over the last 2 decades, cardiac surgery experience with myectomy and mitral plastic surgery, carried out by expert surgeons in "high volume" reference centers of operations performed, have significantly improved patients' symptoms and their prognosis. For this reason, when symptoms and obstruction persist despite optimal medical therapy, myectomy surgery is considered the therapeutic "gold standard" by the guidelines, while septal ablation by injection of small doses of alcohol (ethanol) into the septal artery supplying the hypertrophic area is currently reserved mainly for elderly patients at high surgical risk. The use of dual-chamber pacing has largely fallen into disuse, however, as it has not demonstrated a clear hemodynamic or symptomatic benefit in the majority of patients.
“Myectomy”, explains Professor Franco Cecchi, 'is a complex operation that requires considerable skill and experience, and careful preparation. The narrowing of the flow channel of the left ventricle is due to the thickening of the walls of the heart, which creates an obstruction to the flow of blood out of the heart from the left ventricle. However, over the years it has been understood that it is not only hypertrophy that causes this condition, but mitral valve abnormalities are also responsible. Therefore, the operation is not limited to muscle reduction but also includes mitral plastic surgery, i.e. the remodeling of the mitral valve. This process requires a highly trained surgeon not only in muscle resection, but also in mitral valve repair.'
With the introduction of Mavacamten, already available for "compassionate use" for patients over 18 years of age, and perhaps in the future of Aficamten, there are prospects for long-term treatment, which currently make it more suitable for elderly patients and in the future, perhaps, also in young patients, in whom cardiac surgery currently remains a fundamental solution.
