Centre for Hypertrophic Cardiomyopathy and Valvular Heart Disease

Director: Dr. Paolo Ferrazzi

Organizing Secretary
Tel:(+39) 039-2810598
Email: segreteria.ferrazzi@policlinicodimonza.it
Fax:(+39) 039-2810386
dott paolo ferrazzi
Dott. Paolo Ferrazzi 
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Visit the website www.cardiomiopatiaipertrofica.com

cardiomiopatia ipertroficaThis section of the website is for all those people without medical knowledge who would like to have general information about hypertrophic cardiomyopathy and surgical treatment of this disease. 
Hypertrophic cardiomyopathy is a disease of the heart muscle. The most important manifestation of hypertrophic cardiomyopathy (commonly referred to as HCM) is a marked increase, or thickening, of the heart muscle in general and the left ventricle in particular.  The left ventricle is the portion of the heart that pumps blood into the body.   In the vast majority of patients, this disease is genetic and is usually passed from parent to child.
The structural changes to the heart are variable; some patients may have a very slight increase in muscle thickness, while others may have a muscle thickness that is three times that of normal subjects. 
The progression of HCM is also variable; some patients live a normal life with no symptoms at all, while others may develop heart failure (shortness of breath during mild physical exercise). Others still, may die suddenly and unexpectedly. This enormous difference in presentation and progress makes HCM an extremely difficult disease to treat.  A multidisciplinary approach with the collaboration of cardiologists, surgeons, cardiac imaging specialists, and geneticists is essential.
Thickening of the heart muscle occurs most commonly in the septum, which is the wall that separates the right and left sides of the heart.  In about 20-25% of patients the increase in the thickness of the septum leads to an obstruction to the emptying of the left ventricle (the part of the heart which pumps blood around the body). With exercise, the percentage of patients who develop this obstruction rises to more than 50%.  This obstruction is important because it reduces the amount of blood that goes from the heart to the body.   Abnormalities of the mitral valve (the valve that controls the flow of blood into the left ventricle) may also play an important role in the obstruction.   This form of the disease is called “obstructive hypertrophic cardiomyopathy” and is associated with more severe symptoms and a less favorable outcome.
The majority of patients with obstructive hypertrophic cardiomyopathy develop symptoms of heart failure (shortness of breath on mild effort) and are treated with drugs.  However, a portion of patients do not respond well to drug therapy  and may need an operation known as “myectomy” to relieve the obstruction  (American Heart Association Guidelines and European Society of Cardiology Guidelines for Hypertrophic Cardiomyopathy).
In the myectomy operation the surgeon removes a small portion of muscle from the thickened septum relieving the obstruction and allowing blood to flow freely out of the left ventricle and into the body.  In a portion of patients in whom abnormalities of the mitral valve play an important role in the obstruction, removal of muscle from the thickened septum is not enough to relieve the obstacle to blood flow.  Therefore, in many surgical centers, the mitral valve is replaced with a prosthetic heart valve.  However, a valve prosthesis requires lifetime anticoagulation and is associated with a risk of thromboembolic events, including stroke.  These limitations and potential complications of prosthetic heart valves become particularly important in those many patients with obstructive hypertrophic cardiomyopathy who are young and are expected to have many decades of life in front of them.    
The Myectomy Operation at the Policlinico di Monza, in Italy
Dr. Paolo Ferrazzi is the surgeon that performs the myectomy operation at the Hypertrophic Cardiomyopathy Center of the Policlinico di Monza.  Dr. Ferrazzi’s extensive surgical experience is based on more than 10.000 open-heart operations and documented by his prestigious scientific curriculum.  His particular experience with surgery in hypertrophic cardiomyopathy is reported in recent publications in major international cardiology journals (European Heart Journal  2012, Journal of the American College of Cardiology 2015).  From these publications Dr. Ferrazzi emerges as the surgeon who has performed the highest number of myectomy operations in Europe during the last 5 years, with an operative mortality (< 2%) which is not higher than that of the most renowned surgical centers for hypertrophic cardiomyopathy in North America.
Myectomy associated with mitral valve repair and chordal cutting, a novel operation
During the last three years, a novel operation to relieve the obstruction in patients with hypertrophic cardiomyopathy has been developed by Dr. Ferrazzi at the Policlinico di Monza.  The results of this technique are in press in the Journal of the American College of Cardiology and will be available on Internet from October 13, 2015.  The purpose of this new technique is to relieve the obstruction with a shallow myectomy (resection of a small amount of heart muscle) associated with repair of the abnormalities of the mitral valve.  This technique avoids replacement of the mitral valve with a prosthesis, thus preventing the potential and ominous complications associated with a prosthetic valve.

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