1Petrovsky National Russian Research Center of Surgery, Cardio Surgery of Heart Failure - Moscow - Russian Federation
Background. Obstructive form of hypertrophic cardiomyopathy (HCM) is still the most common inherited myocardial disease characterized by clinical and genetic heterogeneity. Treatment diversity of HCM, including surgical approaches, is available nowdays, but personalized decision-making is still a challenge.
Aim. To review the clinical and diagnostic indications, genetic testing, outcome and 9-years follow up due to optimize surgical decision-making for the HCM patients (pts) with obstructive form.
Material and methods. From 2009 to 2018, 91 symptomatic HCM with obstructive form patients (49 (53,8%) male, the mean age was 47,4±14,8 years) had been operated. 64% pts had III – IV FC NYHA (functional class, New York Heart Association). The average follow-up period after operation was 7 years. Equal clinical, instrumental, biochemical, and genetic investigations were performed for all patients. Histopathological study was carried out for myocardial and valve samplings.
Results: According to the researches, all pts were distinguished in 2 main morphological phenotypes -group I (46 pts, 52%) had predominant hypertrophy of the basal segment of the interventricular septum and group II (42 pts, 48%) had diffuse generalized HCM (diffuse hypertrophy of the septum and free wall, increased number of the hypertrophic papillary muscles displaced to the apex, and significant mitral valve insufficiency). In 25 pts from group I underwent open-heart surgery was performed, and in 21 pts - septal alcohol ablation. Cardioverter-defibrillator (ICD) was implanted for pts with a high risk of sudden cardiac death (SCD). All these pts underwent expanded myoectomy, and parietal resection of the enlarged papillary muscles and left ventricular trabeculae. Chordal sparing mitral valve replacement was performed for 28 of them. All operated pts showed significant cardiac function improvement. During follow-up period in 5 pts was observed ICD chocks, and 2 pts died suddenly due to ventricular tachycardia. Total mutation detection rate was 40% in whole HCM cohort but vary significantly between groups II and I. Histopathological study had revealed mitral valve dysplasia in all studied samples. in
Conclusion: To optimize surgical decision-making for the HCM patients the multidisciplinary approach is needed. Genetic background and fibrosis level may contribute in the SCD risk assessment.