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The unique experience in the treatment of hypertrophic cardiomyopathy in Ukraine

Hypertrophic cardiomyopathy (HCM) is a genetic myocardial disorder characterized by the asymmetric thickening of the walls of the cardiac muscle. Most frequently, the interventricular septum - the structure separating the left and right ventricles of the heart, is thickened. Thus, at the exit from the left ventricle, an obstacle for the movement of blood is formed, and the appropriate symptoms appear.
The unique experience in the treatment of hypertrophic cardiomyopathy in Ukraine

The disorder is inherited according to the autosomal dominant mode of inheritance: this means that the probability of its transmission from mother to child is 50%. Today, the frequency of spread of this pathology in the general population is 1:500, and therefore, taking into account the population of Ukraine, there are approximately 70 thousand people having such a diagnosis in our country. The symptoms of the disorder are quite non-specific: pain in the heart (angor pectoris), shortness of breath, palpitation, fatigue, cardiac arrhythmias, reduced exercise tolerance.

The hazard of HCM is associated with a high risk of sudden cardiac death, especially among young and adolescent children, as well as athletes. Whenever we hear in the news about the sudden death of children in physical education classes, football players on the field, or athletes at competitions, the most common cause of these deaths is HCM, because due to genetic abnormalities, it causes life-threatening arrhythmias such as ventricular tachycardia and ventricular fibrillation.

For the first time it became known about HCM only 50 years ago, when the American scientist Donald Tear described asymmetric hypertrophy (thickening of the cardiac muscle) in adults. Since then, the evolution of approaches to the treatment of this pathology has gone through several stages. Initially, the only and peremptory method was considered a heart transplantation. Later, with the emergence of electrophysiological methods of treatment, such patients were implanted with a two-chamber electric cardiac pacemaker, the effect of which was later compared with the placebo effect by the scientists. The next treatment method was the alcohol septal ablation, a mini-invasive procedure that involves injecting a 96% alcohol solution into the first septal branch of the left coronary artery and creating an artificial heart attack in the area of hypertrophy. However, this procedure, despite its minimally invasive nature (access is performed through the radial artery on the hand), requires strict selection of patients, since HCM is commonly characterized not only by thickening of the interventricular septum, but also by concomitant abnomalies of the mitral subvalvular apparatus, which also require correction.

The unique experience in the treatment of hypertrophic cardiomyopathy in UkraineIn 2016, based at the State Institution “National M.M. Amosov Institute of Cardio-Vascural Surgery Affiliated to National Academy of Medical Sciences of Ukraine”, the program for the prevention of sudden cardiac death was implemented, the main focus of which was the introduction of the latest, unique method of surgical correction of HCM. The author of the technique is considered to be Professor Paolo Ferrazzi (Monza, Italy). He developed a multi-phased operation based on septal myectomy (excision of the thickened interventricular septum) and a set of procedures aimed at correcting abnormalities of the mitral subvalvular apparatus. In Ukraine today, this operation is performed by Doctor of Medical Sciences, Professor Rudenko Kostiantyn Volodymyrovych – a student of Professor P. Ferrazzi. At the moment, he is the only cardiovascular surgeon in the country who performs this procedure.

The surgical correction of HCM occurs in conditions of cardiopulmonary bypass. During the surgery, the heart completely stops and bleeds off, and in the meantime the heart-lung apparatus operates instead. When all the phases of the surgery are completed, the heart-lung apparatus is turned off, and the cardiac resuscitation occurs. On average, such a surgery lasts from 4 to 5 hours, and the rehabilitation process in the hospital lasts no more than a week.

Since 2016, Professor Rudenko K.V. has performed more than 400 such surgeries with a hospital mortality rate of 0.9%, which, according to world-class indicators, allows us to call the Institute an "Expert Center" for the treatment of this pathology. To date, the State Institution "National M.M. Amosov Institute of Cardio-Vascural Surgery Affiliated to National Academy of Medical Sciences of Ukraine" is the second HCM treatment center in Europe in terms of the number of patients who have undergone surgery. About 100-110 such surgeries are performed here per year. For comparison, in Italy, as of the end of 2020, professor Paolo Ferrazzi performed 80 such interventions, and in the United States of America (Mayo Clinic), approximately 100 procedures are performed per year.

It should be noted that the surgical technique of HCM correction requires a multidisciplinary approach and high qualification of the operating surgeon. The professional team treating a single patient consists not only of a cardiac surgeon, but also interventional cardiologists, radiologists, functional diagnostics doctors and cardiologists. Despite the fact that the method is quite invasive, since access is performed through an incision on the chest. It has a number of significant advantages: no disease recurrence and the possibility of 100% correction of all phenotypic disease manifestations. But given the current trends towards mini-accesses and performing cardiac surgery through a small incision, this year we plan to improve the Ferrazzi surgery and perform it through a mini-thoracotomy. This will significantly reduce the time spent by patients in the hospital and speed up their rehabilitation period, since when using this access, the sternum is not damaged.

Lazoryshynets Vasyl
Academician, Director of the National M.M. Amosov Institute of Cardio-Vascural Surgery Affiliated to National Academy of Medical Sciences of Ukraine
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