Aim : To study morphofunctional changes of the heart, genetic testing, the indications and long-term results of surgical treatment of patients with hypertrophic cardiomyopathy (HCM) with multilevel obstruction.
Material and methods : From 2009 to 2017 a total of 124 patients (pts) with HCM (63 women, 61 men) were analyzed. The mean age was 53±2,8 years. 79 (64%) pts had III – IV FC NYHA (functional class, New York Heart Association). According to ECHO, there was found a significant increase of the thickness of interventricular septum (IVS) (2,24±0,03 sm,p<0.05), increase of the gradient at left ventricle (LV) outflow tract (OP) (71,5±6.9 mm hg,p<0.05) and unreliable decrease in end-systolic volume (ESVLV) (31,3± 2.6 ml). According to MRI/MCT of the heart in 77pts there was registered increased volume of the anterior and posterior papillary muscles, to their dystopia with reduction of ESVLV and significantly increased myocardial mass of LV in diastole (311±45gr;p<0.05), which we determined as a diffuse-generalized form of HCM; in 22 pts – asymmetric form of HCM. 63 pts with the obstructive form of HCM underwent operation of the extended myectomy with parietal resection of the papillary muscles in conditions of open heart surgery (included 42 pts with replacement of the mitral valve), 5 – endovascular ablation of septal perforator, 27 - implantation of cardioverter defibrillator (ICD), 39 – conservative therapy.
Results : In pts after open heart surgery found a significant improvement in a 1, 3, 5 and 7 years after surgery: I FC (NYHA) – 34 (57%) patients, of FC II (NYHA) – in 25 (53%) patients. Triggering ICD was observed in 5 (19%) pts in the postoperative period. 4 patients (6,3%) died due to life-threatening arrhythmias. ECHO revealed a significant decrease in thickness of IVS (from 2.27±0.02 to 1.5±0.02 cm, p<0.05) and the gradient at OPLV (71,38±6.7 to 4,75±1.3 mm hg, p<0.05), the false increase of ESVLV (28,31±2.8 ml to 34.4±2,9ml p>0.05). Classic "sarcomeric" genetic mutations were surprisingly only found in 14% pts, in86% pts were found other genetic disorders.
Conclusion : Reconstructive extended myectomy with the parietal resection of papillary muscle and mitral valve replacement with chordal preservation provides several long-term benefits in pts with HCM. Many questions remain unanswered about the phenotype-genotype correlations and the prognostic value of particular mutations in different genes.