General Description of the Center
Cardiomyopathy Center is the only medical center in Romania and one of the 5 centers in Europe that deals with the diagnosis and treatment of obstructive hypertrophic cardiomyopathy. Affiliated to the Italian center, the Cardiomyopathy Center benefits from the expertise of over 500 operated cases.
The Center makes available for all patients dedicated specialists that make possible the performance of all clinical and paraclinical examinations required by the diagnosis, but also the genetic test, which is extremely important in cases with transmission within the family.
The Cardiomyopathy Center is led by Dr. Lucian Florin Dorobanţu and benefits from the expertise of Professor Dr. Paolo Ferrazzi in the surgical treatment of the obstructive hypertrophic cardiomyopathy.
The surgical team of the Center approaches an original operative technique, proposed by Prof. P. Ferrazzi and internationally acknowledged, which, unlike the classical approach, brings in the benefit of preserving the mitral valve, with a success rate of over 99% (the highest rate of mitral valve preservation in the world).
About hypertrophic cardiomyopathy
Hypertrophic cardiomyopathy (HCM) is a primary heart disease determined in most cases by genetic factors, characterized by thickening of the ventricular walls and disorganization of the architecture of the heart muscle. This is the most frequent cardiomyopathy, with a prevalence of 0.2% of the general population.
Out of the patients with HCM, an important proportion of approximately 30% develop its obstructive form, called obstructive hypertrophic cardiomyopathy (HOCM), which is due to the fact that, at rest or during a physiological challenge (Valsalva maneuver), in the ejection tract of the left ventricle appears a pressure gradient. If the pressure is higher than 50 mmHg, the obstruction is considered hemodynamically important.
Ventricular hypertrophy is frequently associated with anomalies of the mitral system, presenting the mitral failure, severe more often than not, which contributes to the dynamic obstruction in the ventricle. Sometimes this is the main obstruction mechanism in the ejection tract – the case of patients with relatively thin septum.
Clinically speaking, the symptomatology varies widely, from asymptomatic up to invalidating cases, with severe cardiac failure or sudden death. HOCM is one of the main causes of sudden death in young patients, especially in performing athletes.
Beside the physical examination, the evaluation of these patients presupposes carrying put the following investigations: electrocardiogram, transthoracic echocardiography at rest and under stress, transesophageal echocardiography, Holter ECG/48 hours, cardiac magnetic resonance (which is essential even in the intraoperative management of patients) and, in most cases, genetic testing.
The treatment envisages symptomatic patients. It may be represented by medication, which is effective in most cases; in the treatment resistant ones, invasive treatment is the recourse of election.
According to American and European guides, the recommended invasive therapy is the surgical one; according to the same guides, due to its superior results, it must be carried out in expert centers only.
The surgical treatment is at this moment a class I indication and it consists of extended septal myectomy or the Morrow operation.
Myectomy presupposes an extended muscular resection in the basal septum in order to sufficiently widen the ejection tract of the left ventricle and thereby reduce the SAM (systolic anterior motion) of the mitral valve.
At Monza Hospital we use the “one piece” myectomy technique, which presupposes the resection of the septum in a single piece, whose dimensions are strictly calculated and achieved individually, for each and every patient. Technically difficult, it may be achieved only after a long experience and it is based on the data provided by the echocardiography and cardiac magnetic resonance.
There is also an important share of patients with a significant intraventricular gradient, who present a moderate septum hypertrophy, which in most cases associates anomalies of the mitral system that contributes to the obstruction of the ejection tract.
The team led by Prof. Paolo Ferazzi, which has by far the greatest expertise in Europe in treating HOCM, has established the importance of secondary mitral chordae in the mitral failure of HOCM. The resection of secondary mitral chordae associated to myectomy, along with the mobilization of papillary muscles, reduces VMA tethering and pushes the VMA coaptation point to the posterior side, thereby eliminating VMA from the ejection tract.
This technique, published in 2015 in the ”Journal of the American College of Cardiology”, the most prestigious specialized magazine in the world, underlines once again the idea expressed in the latest European and American guides with regard to HOCM, namely that the management of this disease is to be carried out by specialized teams. The complex investigative protocol, with dedicated cardiologists, with radiologists specialized in cardiac MRI, coordinated by specialized surgeons, and defining elements of an adequate and modern treatment.
The benefits for the patients treated by these techniques are: the disappearance of symptoms and an important reduction of the risk of sudden death, risk of postoperative mortality similar to the general population, and stable results on the long term, the patient getting back to a normal life.
When surgery cannot take place, in case of patients with important comorbidities and with high surgical risk, alcohol septum ablation is recommended. It has been proven that this technique requires a favorable anatomy and it does not solve mitral failure, determining more frequent complications in terms of residual gradient in ejection tract of the left ventricle, of incidence of bundle block or complete atrioventricular block. Therefore, in our opinion, it is reserved today for inoperable cases.