MITRAL VALVE PROLAPSE: A SMALL ANOMALY OF THE HEART, A NONSPECIFIC MANIFESTATION OF HEREDITARY CONNECTIVE TISSUE DISORDERS OR AN INDEPENDENT SYNDROME?

The review article presents modern concepts of the mitral valve prolapse. The issues of pathogenesis, diagnostic criteria of primary and probable prolapse are reflected, the issues of epidemiology are considered in detail. The problems associated with significant overdiagnosis of this condition in o...

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Main Authors: E. V. Timofeev, E. G. Malev, E. V. Zemtsovsky, S. V. Reeva
Format: Article
Language:English
Published: Scientia Publishing House 2022-02-01
Series:Juvenis Scientia
Subjects:
Online Access:https://jscientia.org/index.php/js/user/setLocale/en_US?source=/index.php/js/article/view/135?utm_source=DOAJ
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author E. V. Timofeev
E. G. Malev
E. V. Zemtsovsky
S. V. Reeva
author_facet E. V. Timofeev
E. G. Malev
E. V. Zemtsovsky
S. V. Reeva
author_sort E. V. Timofeev
collection DOAJ
description The review article presents modern concepts of the mitral valve prolapse. The issues of pathogenesis, diagnostic criteria of primary and probable prolapse are reflected, the issues of epidemiology are considered in detail. The problems associated with significant overdiagnosis of this condition in our country (up to 36.8%) are noted, which is due to ignoring internationally agreed algorithms and incorrect interpretation of the results obtained during echocardiography. When using a parasternal two-chamber position and choosing a diagnostic threshold of 3 mm or more, mitral valve prolapse is detected in 4.3% of practically healthy young people, which is comparable to population-based world studies, in the absence of sex differences. The place of mitral valve prolapse in the algorithms for diagnosing hereditary syndromes — Marfan and Ehlers-Danlos, its pleiotropy, which should be taken into account when stratifying the risk of clinically significant events and in case of probable prolapse, is discussed. The situations that allow to interpret mitral valve prolapse as an independent hereditary syndrome or a small anomaly of the heart are indicated. The article presents current views on the pathogenesis of mitral valve prolapse, the role of transforming growth factor-β in the progression of myxomatous degeneration of the valves, the development of manifestations of cardiomyopathy. The prognostic value of primary prolapse in its natural course is shown — the progression of myxomatosis of the valves, mitral insufficiency, dilation of the main vessels.
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spelling doaj.art-fb4d512fa7a041a39734c675507a02022022-12-21T23:29:50ZengScientia Publishing HouseJuvenis Scientia2414-37822414-37902022-02-018151810.32415/jscientia_2022_8_1_5-18MITRAL VALVE PROLAPSE: A SMALL ANOMALY OF THE HEART, A NONSPECIFIC MANIFESTATION OF HEREDITARY CONNECTIVE TISSUE DISORDERS OR AN INDEPENDENT SYNDROME?E. V. Timofeev0https://orcid.org/0000-0001-9607-4028E. G. Malev1https://orcid.org/0000-0002-6168-8895E. V. Zemtsovsky2https://orcid.org/0000-0003-2775-5158S. V. Reeva3https://orcid.org/0000-0002-9183-2245Saint Petersburg State Pediatric Medical UniversitySaint Petersburg State Pediatric Medical UniversitySaint Petersburg State Pediatric Medical UniversitySaint Petersburg State Pediatric Medical UniversityThe review article presents modern concepts of the mitral valve prolapse. The issues of pathogenesis, diagnostic criteria of primary and probable prolapse are reflected, the issues of epidemiology are considered in detail. The problems associated with significant overdiagnosis of this condition in our country (up to 36.8%) are noted, which is due to ignoring internationally agreed algorithms and incorrect interpretation of the results obtained during echocardiography. When using a parasternal two-chamber position and choosing a diagnostic threshold of 3 mm or more, mitral valve prolapse is detected in 4.3% of practically healthy young people, which is comparable to population-based world studies, in the absence of sex differences. The place of mitral valve prolapse in the algorithms for diagnosing hereditary syndromes — Marfan and Ehlers-Danlos, its pleiotropy, which should be taken into account when stratifying the risk of clinically significant events and in case of probable prolapse, is discussed. The situations that allow to interpret mitral valve prolapse as an independent hereditary syndrome or a small anomaly of the heart are indicated. The article presents current views on the pathogenesis of mitral valve prolapse, the role of transforming growth factor-β in the progression of myxomatous degeneration of the valves, the development of manifestations of cardiomyopathy. The prognostic value of primary prolapse in its natural course is shown — the progression of myxomatosis of the valves, mitral insufficiency, dilation of the main vessels.https://jscientia.org/index.php/js/user/setLocale/en_US?source=/index.php/js/article/view/135?utm_source=DOAJmitral valve prolapseprevalencebarlow's diseasefibroelastic deficiencyhereditary disorders (dysplasia) of connective tissuesmall heart abnormalitiesmarfan syndromeehlers-danlos syndromeloeys-dietz syndromestickler syndrome
spellingShingle E. V. Timofeev
E. G. Malev
E. V. Zemtsovsky
S. V. Reeva
MITRAL VALVE PROLAPSE: A SMALL ANOMALY OF THE HEART, A NONSPECIFIC MANIFESTATION OF HEREDITARY CONNECTIVE TISSUE DISORDERS OR AN INDEPENDENT SYNDROME?
Juvenis Scientia
mitral valve prolapse
prevalence
barlow's disease
fibroelastic deficiency
hereditary disorders (dysplasia) of connective tissue
small heart abnormalities
marfan syndrome
ehlers-danlos syndrome
loeys-dietz syndrome
stickler syndrome
title MITRAL VALVE PROLAPSE: A SMALL ANOMALY OF THE HEART, A NONSPECIFIC MANIFESTATION OF HEREDITARY CONNECTIVE TISSUE DISORDERS OR AN INDEPENDENT SYNDROME?
title_full MITRAL VALVE PROLAPSE: A SMALL ANOMALY OF THE HEART, A NONSPECIFIC MANIFESTATION OF HEREDITARY CONNECTIVE TISSUE DISORDERS OR AN INDEPENDENT SYNDROME?
title_fullStr MITRAL VALVE PROLAPSE: A SMALL ANOMALY OF THE HEART, A NONSPECIFIC MANIFESTATION OF HEREDITARY CONNECTIVE TISSUE DISORDERS OR AN INDEPENDENT SYNDROME?
title_full_unstemmed MITRAL VALVE PROLAPSE: A SMALL ANOMALY OF THE HEART, A NONSPECIFIC MANIFESTATION OF HEREDITARY CONNECTIVE TISSUE DISORDERS OR AN INDEPENDENT SYNDROME?
title_short MITRAL VALVE PROLAPSE: A SMALL ANOMALY OF THE HEART, A NONSPECIFIC MANIFESTATION OF HEREDITARY CONNECTIVE TISSUE DISORDERS OR AN INDEPENDENT SYNDROME?
title_sort mitral valve prolapse a small anomaly of the heart a nonspecific manifestation of hereditary connective tissue disorders or an independent syndrome
topic mitral valve prolapse
prevalence
barlow's disease
fibroelastic deficiency
hereditary disorders (dysplasia) of connective tissue
small heart abnormalities
marfan syndrome
ehlers-danlos syndrome
loeys-dietz syndrome
stickler syndrome
url https://jscientia.org/index.php/js/user/setLocale/en_US?source=/index.php/js/article/view/135?utm_source=DOAJ
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AT egmalev mitralvalveprolapseasmallanomalyoftheheartanonspecificmanifestationofhereditaryconnectivetissuedisordersoranindependentsyndrome
AT evzemtsovsky mitralvalveprolapseasmallanomalyoftheheartanonspecificmanifestationofhereditaryconnectivetissuedisordersoranindependentsyndrome
AT svreeva mitralvalveprolapseasmallanomalyoftheheartanonspecificmanifestationofhereditaryconnectivetissuedisordersoranindependentsyndrome