OBJECTIVES We studied 16 patients after failed alcohol septal ablation who underwent extended septal myectomy to analyse the results of surgical correction and identify technical pitfalls the surgeons may be confronted by. METHODS Between October 2017 and March 2019, 16 patients underwent surgical extended septal myectomy with accompanying anomalous secondary chordae resection, papillary muscles mobilization [in 9 (56.3%) patients], and anterior mitral leaflet plication after previously failed alcohol septal ablation. Routine preoperative computed tomography or cardiac magnetic resonance planning and intraoperative transoesophageal echocardiography were performed in each of the studied patients. Major technical features were identified and complemented during septal myectomy of the calcified interventricular septum. RESULTS The mean age of the studied patients accounted 50.5 ± 14.6, median—54; males—5 (31.3%). Mean cross-clamp time accounted 52 ± 7.2 min. Calcified basal interventricular septum was identified in 2 (12.5%) patients. No iatrogenic ventricular septal defect (0%) was made during surgical correction. Peak systolic pressure gradient decreased from 86 (interquartile range: 75–104.7) to 20 (16–22) mmHg (P< 0.001). No patients with moderate or severe mitral regurgitation were identified, whereas before the procedure, the number of those accounted 13 (81.2%) individuals. In-hospital and overall mortality after septal myectomy accounted 0%. CONCLUSIONS Extended septal myectomy in patients who previously underwent alcohol septal ablation is a safe procedure that affects all pathological manifestations of the disease. Routine preoperative computed tomography or cardiac magnetic resonance provides detailed anatomy of the anomalous left ventricle and subvalvular structures and allows to measure the extension of myectomy preventing the occurrence of iatrogenic ventricular septal defect. Septal myectomy of the calcified interventricular septum requires avoidance of ‘one-piece technique’ since fragmental myectomy allows visually control the adequacy of the left ventricle outflow tract release.

Fabry disease (FD) is an X-linked lysosomal storage disease caused by a mutation in the gene encoding α-galactosidase A and leads to reduced activity or complete absence of this enzyme, which causes the accumulation of globotriaosylceramide (Gb3) and its deacylated form (lyso-Gb3) in cells of the whole body. FD can occur both with multisystem manifestations, including damage to the nervous system, kidneys, and skin, and can affect only the heart. Cardiac involvement is a major cause of poor quality of life and death in patients with FD and an underrecognized cause of heart failure with preserved ejection fraction and ventricular arrhythmias in men over 30 years of age and women over 40 years of age. Cardiac damage begins at an early age, progresses subclinically until the appearance of significant symptoms, and usually manifests as leftventricular hypertrophy, mimicking hypertrophic cardiomyopathy. After the introduction of enzyme replacement therapy, early recognition of FD and differential diagnosis with other causes of leftventricular hypertrophy have become crucial to limit the progression of the disease. Recent advances in the understanding of cardiac pathophysiology and imaging have improved diagnostic and therapeutic approaches to the cardiac manifestations of this pathology. Modern achievements in the study of cardiac manifestations of FD have made it possible to significantly improve diagnostic and therapeutic approaches, in particular, in relation to the identification of pathogenetic mechanisms of organ damage and early disruption of their function. A better understanding of secondary pathogenic pathways, such as myocardial inflammation, may influence future therapeutic strategies and timely diagnosis of FD. Delay in diagnosis and untimely initiation of treatment remain critical problems for many patients with FD, especially for patients with late-onset cardiovascular manifestations, in whom treatment effects may be more limited and ineffective. Cooperation between specialists in genetic diseases and cardiologists remains important to identify patients before the appearance of cardiac symptoms in order to obtain maximum therapeutic effects.

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Objective. To study the immediate and remote follow-up results of treatment in patients, suffering obstructive hypertrophic cardiomyopathy and concurrent ischemic heart disease, using the alcohol septal ablation in combination with simultaneous endoprosthesis of coronary arteries. Materials and methods. In the investigation were included 129 patients, suffering obstructive hypertrophic cardiomyopathy, to whom the alcohol septal ablation was performed in 2009 - 2018 yrs in M. M. Amosov National Institute of Cardiovascular Surgery. All the patients were distributed into two groups: the first -14 (10.9%) patients with concurrent ischemic heart disease and the second -115 (89.1%) patients without concurrent ischemic heart disease. Results. Reduction of the systolic pressure gradient in the exit tract of the left ventriculus, mitral regurgitation, and the functional class characteristic in accordance to criteria of a New-York Association of Cardiologists in both groups in immediate and late periods of observation have appeared statistically proved. In a remote period of follow-up in 13 (92.9%) patients, suffering the ischemic heart disease, a satisfactory hemodynamical result was registered, and in 1 (7.1%) - poor. Conditionally poor results in this group of patients were absent. The patients without an ischemic heart disease (n=107) in accordance to the above mentioned indices were distributed in a follow manner: 74 (69.2%), 28 (26.2%) and 5 (4.7%), accordingly. Statistically significant difference in accordance to hemodynamical results between two groups of patients was absent in immediate and remote periods of follow-up. Conclusion. Simultaneous conduction of the alcohol septal ablation in combination with endoprosthesis of coronary arteries in patients, suffering obstructive hypertrophic cardiomyopathy and concurrent ischemic heart disease, constitutes a safe proved combined intervention procedure, which owes good immediate results, persisting in the remote period.

The aim — to investigate the effectiveness of surgical septal myectomy in patients with obstructive form of hypertrophic cardiomyopathy (HCM) and to determine its effect on survival and quality of life.Materials and methods. The research included 118 consequential symptomatic patients with obstructive form of HCM who underwent surgical extensive myectomy with secondary chordae resection and mobilization of the anterior and posterior groups of papillary muscles (PM). An evaluation of the following parameters in group of patients before and after surgical intervention was performed: systolic pressure gradient (SPG) on the left ventricle outflow tract (LVOT), mitral regurgitation (MR) degree, NYHA functional class, survival rate and main post­operative complications.Results and discussion. According to the obtained data, SPG decreased from 93.6 ± 23.2 mmHg prior the surgery to 19.7 ± 11.4 mmHg after the treatment (p < 0.001). 21 (17.8 %) patients had moderate degree of MR after the surgery, while before the intervention the number of patients who had moderate or severe MR degree made up 101 (85.5 %) out of 118 (p < 0.001). Out of 118 patients, 36 (30.5 %) of whom had III — IV NYHA functional class of heart failure before the procedure, 115 (97.4 %) switched to functional class I — II in the latest follow­up (p < 0.001). The mortality accounted for 1.7 %. Out of 118 patients under study, one (0.8 %) underwent cardioverter­defibrillator implantation in the post­operative period for the prophilaxis of sudden death, and 5 (4.2 %) patients underwent pacemaker implantation due to complete post­operative AV­block.Conclusions. Surgical septal myectomy by professor P. Ferrazzi procedure is a gold standard of treatment of patients with obstructive form of HCM. Successful correction of HCM can be conducted only by a high­qualified surgeon with experience of reconstruction of valvular pathologies and in an expert center, where it is possible to perform routine pre­operative MRI or CT‑planning and mandatory intraoperative echocardiographic control.