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  wit...

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Main Authors: D. V. Bogdanov, I. I. Shaposhnik
Format: Article
Language:Russian
Published: «FIRMA «SILICEA» LLC 2019-11-01
Series:Российский кардиологический журнал
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Online Access:https://russjcardiol.elpub.ru/jour/article/view/3489
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Summary: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.
ISSN:1560-4071
2618-7620