Clinical and demographic characteristics, prevalence of cardiometabolic risk factors and peculiarities of the clinical course of hypertrophic cardiomyopathy in different age groups. Part one

Мұқаба

Дәйексөз келтіру

Толық мәтін

Ашық рұқсат Ашық рұқсат
Рұқсат жабық Рұқсат берілді
Рұқсат жабық Рұқсат ақылы немесе тек жазылушылар үшін

Аннотация

Hypertrophic cardiomyopathy (HCM) is the most common hereditary myocardial disease with evident anatomical, clinical and genetic heterogeneity.

The aim: to demonstrate the diagnostic criteria for HCM in patients with and without concomitant arterial hypertension (HCM and HCM + AH), as well as the main elements of differential diagnosis in case of clinical suspicion to phenocopies / specific causes of HCM.

Material and methods. 249 patients with the HCM phenotype were examined. Diagnosis of HCM was made in accordance with Russian clinical guidelines for HCM 2025. The criterion for HCM in adults was maximum wall thickness of the left and/or right ventricle ≥ 1.5 cm, the criterion for HCM with concomitant AH was ≥ 20 mm. When LV wall thickness in patients with HCM and concomitant arterial hypertension was found to be in the “gray zone” (15–19 mm), the conclusion about probable HCM was made basing on a thorough analysis of additional characteristics of AH and HCM phenotype.

Results. There were no cases of Fabry disease among the examined patients. In 7 participants with HCM phenotype aged 60 years and older, cardiac forms of amyloidosis were diagnosed in 6.2% of cases (2.8% of cases among all examined patients). 15 patients with a wall thickness from 15 to 19 mm in the absence of additional characteristics of arterial hypertension and HCM phenotype and 22 patients with HCM with an unspecified hereditary factor were referred for additional examination (magnetic resonance imaging with contrast and, accordingly, examination of the proband’s family members in the 2nd–3rd generations). HCM with a specified heredity factor was verified in 205 patients aged 18 to 87 years (58 [42; 65]) and was represented by the categories of HCM (34.6%) and HCM + AH (65.4%). Criteria for diagnosing HCM with and without AH were followed. In the group as a whole, the incidence of AH in case of non-familial HCM reached 81.3%, in non-familial HCM in the age category ≥ 45 years – 84.8%.

Conclusion. In patients aged 60 years and older, cardiac forms of amyloidosis were diagnosed by us in 6.2% of cases. The overwhelming majority of patients with HCM phenotype were diagnosed with familial and non-familial HCM. In general, the cohort of patients with HCM + AH in combination with other cardiometabolic risk factors and without them accounted for 65.4%.

Толық мәтін

Рұқсат жабық

Авторлар туралы

Alexandra Gudkova

Pavlov University; Almazov National Medical Research Centre

Email: alexagood-1954@mail.ru
ORCID iD: 0000-0003-0156-8821
SPIN-код: 7246-7349

MD, Dr. Sci. (Medicine), professor of the Department of faculty therapy with a course in endocrinology, cardiology with the clinic named after academician G.F. Lang, head of the Department of cardiomyopathies of the Research Institute of Cardiovascular Diseases of Scientific and Clinical Research Center, leading researcher at the Institute of Molecular Biology and Genetics

Ресей, Saint Petersburg; Saint Petersburg

Tinatin Bezhanishvili

Pavlov University

Хат алмасуға жауапты Автор.
Email: tinatin93@rambler.ru
ORCID iD: 0000-0002-3167-6340
SPIN-код: 7888-2100

MD, assistant at the Department of faculty therapy with a course in endocrinology, cardiology with the clinic named after academician G.F. Lang, junior researcher at the Department of cardiomyopathies, Research Institute of Cardiovascular Diseases of Scientific and Clinical Research Center

Ресей, Saint Petersburg

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2. 1. Study design and diagnostic criteria for hypertrophic cardiomyopathy in certain categories of patients: with hypertrophic cardiomyopathy and its combination with arterial hypertension

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3. 2. "Red flags" of phenotypes and specific causes of hypertrophic cardiomyopathy

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