Clinical variants, outcomes and prognosis of hypertrophic non-obstructive cardiomyopathy: results of long-term follow-up

Aim. Based  on the results  of a long-term  follow-up, to determine  the clinical variants  of  hypertrophic  non-obstructive cardiomyopathy  (HNOCM) and  develop criteria for the progression of the disease.

Material and methods. The results of long-term follow-up were evaluated  for 97 patients  with HNOCM, men — 57 (58,8%), women — 40 (41,2%), average  age — 42,5±1,52  years  (M±σ). The  average  follow-up was  11,0±5,73  years.  Research methods   were  physical  examination,  electrocardiography (ECG), 24-hour  ECG monitoring with assessment of heart  rate  variability, Doppler  echocardiography (echo).   Physical  and  instrumental   re-examination   of  patients   was  used   for determining of changes.

Results.  Clinical progression was  detected in 46  (47,4%)  patients.   The  most common progression variants were deterioration  of chronic heart failure (54,3%), cardiac arrhythmias (45,7%) and a combined  one (37%). The aggravation of echo data was found in 43 (44,3%) patients. The most frequent progression variants werecombination of different dynamics scenarios (79,1%), including a decrease in the mobility of left ventricle walls (67,4%), an increase  of left atrium dimension (58,1%), and diastolic dysfunction deterioration  (67,4%). Aggravation of myocardial hypertrophy  was noted  in only 3 cases. In 5 (5,15%) cases, the dilation stage  of HNOCM was recorded. A  scoring  model for assessing of progression risk were proposed. It includes  the duration of HNOCM >20 years,  baseline  ≥II FC chronic heart  failure, ≥2 clinical manifestations  of the disease, left ventricular myocardialmass index >200 g/m² , left atrial volume index >34,0 ml/m² , cardiac output <5 l/min, decrease in heart rate variability.

Conclusion.  HNOCM is a disease with a complicated  prognosis, progressing in 64% of patients, more often with a long course.  The clinical progression of HNOCM was manifested  by the diverse dynamics of complaints.  Structural  and functional progression was manifested mainly by a combination of different dynamics variants (in 79,1% of cases), including a decrease in the mobility of left ventricular walls, an increase  of left atrium  dimension,  aggravation  of diastolic  dysfunction,  but  not deterioration  of myocardial hypertrophy. The main criteria for progression: disease duration >20 years, baseline ≥II FC chronic heart failure, ≥2 clinical manifestations of the  disease, increase  of left ventricular myocardial mass  index and  left atrial volume index, decrease of cardiac output and heart rate variability.

1. Elliott P. M. Anastasakis A, Borger MA et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy. Eur Heart J. 2014;35(39):2733-79. doi:10.1093/eurheartj/ehu284.

2. O’Mahony C, Jichi F, Pavlou M, et al. Hypertrophic Cardiomyopathy Outcomes Investigators. A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM Risk-SCD). Eur Heart J. 2014;35,30:2010-20. doi:10.1093/eurheartj/eht439.

3. Belenkov YuN, Privalova EV, Kaplunova VYu. Hypertrophic Cardiomyopathy. M.: GEHOTAR Media, 2011. р. 392. (In Russ.)

4. Maron BJ, Rowin EJ, Casey SA, et al. Risk stratification and outcome of patients with hypertrophic cardiomyopathy >=60 years of age. Circulation. 2013;127,5:585-93. doi:10.1161/CIRCULATIONAHA.112.136085.

5. Komissarova SM. Primary hypertrophic cardiomyopathy: frequency of occurrence in the Republic of Belarus, course options, choice of therapy, prediction determination. Kardiologiya v Belarusi. 2009;1(02):64-74. (In Russ.)

6. Komissarova SM, Vajhanskaya TG, Shket AP. National recommendations “Diagnosis and treatment of primary hypertrophic cardiomyopathy” Minsk.: 2010. р. 36. (In Russ.)

7. Bogdanov DV, Bolotov AA. Differential diagnosis of hypertrophic non-obstructive cardiomyopathy. Modern problems of science and education. 2014;6. (In Russ.) http://www.science-education.ru/ru/article/view?id=16570 (11 september 2019).

8. Bogdanov DV. Variants of the course and predictors of disease progression in hypertrophic non-obstructive cardiomyopathy. Modern problems of science and education. 2015;5. (In Russ.) http://www.science-education.ru/ru/article/view?id=21915 (11 september 2019).

9. Bogdanov DV, Shaposhnik II. Doctor’s tactics for hypertrophic nonobstructive cardiomyopathy. Clin. Pharmacol. Ther., 2017;26(3):31-6. (In Russ.)

10. Mareev VYu, Ageev FT, Arutyunov GP, et al. National recommendations of OSSN, RKO and RNMOT for the diagnosis and treatment of heart failure (fourth revision). Russian Heart Failure Journal. 2013;14(7):379-472. (In Russ.) doi:10.18087/rhfj.2013.7.1860.

11. Hancock EW, Deal BJ, Mirvis DM, et al. AHA/ACCF/HRS Recommendations for the Standardization and Interpretation of the Electrocardiogram. Part V: Electrocardiogram Changes Associated With Cardiac Chamber Hypertrophy. A Scientific Statement From the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society. JACC. 2009;53,11:992-1002. doi:10.1016/j.jacc.2008.12.015.

12. Makarov LM, Komolyatova VN, Kupriyanova OA, et al. National Russian guidelines on application of the methods of holter monitoring in clinical practice. Russian Journal of Cardiology. 2014;(2):6-71. (In Russ.) doi:10.15829/1560-4071-2014-2-6-71.

13. Rybakova MK. Echocardiography from M. K. Rybakova with DVD 2nd edition. M.: Izdatelskij dom Vidar-M. 2018. Р. 600. (In Russ.)

14. Recommendations for quantifying the structure and function of the heart chambers. Russ J Cardiol. 2012;3(95):28-54. doi:10.15829/1560-4071-2012-4s4-1-27.

15. Goto D, Kinugawa S, Hamaguchi S, et al. Clinical characteristics and outcomes of dilated phase of hypertrophic cardiomyopathy: report from the registry data in Japan. J Cardiol. 2013;61,1:65-70. doi:10.1016/j.jjcc.2012.08.010.