Masterclass on Hypertrophic Obstructive Cardiomyopathy
by Prof. Maurizio Pieroni and Dr. Michele Ciabatti
On November 26 and 27, 2024, an important masterclass on the diagnosis, treatment and management of hypertrophic obstructive cardiomyopathy (HOC) was held in Florence. This condition affects a significant subgroup of patients affected by HOC and determines a significant impact in terms of morbidity, mortality and reduction in quality of life in this population. The event saw the participation of a large group of experts in the field, with the possibility of providing a global and in-depth vision of the various topics concerning the disease.
At the diagnostic and prognostic level, the new possibilities offered by modern imaging technologies were discussed (cardiac magnetic resonance imaging, echocardiographic strain analysis) and their impact on clinical practice. Another point of discussion was the role of the cardiopulmonary test in defining the functional capacity but also the prognosis in this pathology. This test allows a global evaluation of the interactions between the heart, lungs and skeletal muscle, and therefore offers a complete vision of the functional impact of the disease on the patient, a fact often underestimated by common cardiological investigation methods.
Another extremely important theme was that of the genetic test and the complex analysis work required for a correct interpretation of the results. In fact, given the enormous amount of information deriving from gene sequencing, it is becoming increasingly essential to obtain genetic and cardiological counseling, which allows for a careful and multiparametric evaluation of the results to avoid misleading readings.
At the therapeutic level, a large space has been dedicated to the role of myosin inhibitors, a new class of drugs able to act on the molecular mechanisms of the disease. These molecules have shown a significant improvement in symptoms, echocardiographic parameters and laboratory markers in patients with CMIO. In the future, they could also modify the natural history of the disease.
The biological phenomena implicated in the development of hypertrophic cardiomyopathy were also discussed, knowledge of which is essential to direct therapy and develop new dedicated therapeutic approaches in the future.
Interactive sessions in the field ofechocardiography and cardiac magnetic resonance imaging and genetics represented a valuable opportunity for practical interaction between experts in the field, participants and patients in order to apply the new knowledge in clinical practice. The presence of some volunteer patients during the echocardiography sessions allowed us to delve deeper into the point of view of the patient affected by CMIO. This aspect was also the subject of discussion in the sessions dedicated to the "journey" that the patient faces during his condition (the so-called "patient journey") and the role that patient associations, such as AICARM, can have in clinical and psychological support and in research activities in the field of cardiomyopathies.
This event therefore represented a precious moment of dissemination, updating and discussion of the state of the art on CMIO, with an eye to future prospects.
The organization of other similar events on this topic and other cardiomyopathies can certainly favor the development of a better awareness and culture of the cardiologists involved on these pathologies in order to provide the best possible management for the affected patients and their families.
Prof. Maurizio Pieroni
Associate Professor of Cardiovascular Diseases at the University of Florence, former head of the Cardiomyopathy and Rare Cardiac Diseases Outpatient Clinic, San Donato Hospital in Arezzo.
He graduated cum laude from the Catholic University in 1997, obtaining the specialization in Cardiology cum laude in 2001 at the same institution. He then worked for 2 years in the intensive care unit of the San Raffaele Hospital in Milan, before returning to the Catholic University in 2004. In 2005 he also obtained a PhD in human pathology. From 2006 to 2011 he was head of the Cardiomyopathy Unit at the Catholic University Policlinico Gemelli in Rome. In 2011 he moved to the Hospital of Arezzo to direct a new Cardiomyopathy Unit and in 2024 he took up the chair at the University of Florence.
Professor Pieroni has been interested in the study of cardiac pathology and cardiomyopathies, in particular hypertrophic cardiomyopathy, Fabry disease and myocarditis, since he was a medical student.
He is an expert in performing and histologically evaluating endomyocardial biopsies, as well as interpreting echocardiography and cardiac magnetic resonance imaging in the field of cardiomyopathies. He is deeply involved in both research and patient management in the field of inherited cardiomyopathies, lysosomal storage disorders, and channelopathies.
His current research in hypertrophic cardiomyopathy focuses on the differential diagnosis of phenocopies and the identification of mechanisms of disease progression and clinical instability, including systemic and myocardial inflammation.
