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Common presentation of rare diseases: Left ventricular hypertrophy and diastolic dysfunction

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F18%3A10376610" target="_blank" >RIV/00216208:11110/18:10376610 - isvavai.cz</a>

  • Alternative codes found

    RIV/00064165:_____/18:10376610

  • Result on the web

    <a href="https://doi.org/10.1016/j.ijcard.2018.01.006" target="_blank" >https://doi.org/10.1016/j.ijcard.2018.01.006</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1016/j.ijcard.2018.01.006" target="_blank" >10.1016/j.ijcard.2018.01.006</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Common presentation of rare diseases: Left ventricular hypertrophy and diastolic dysfunction

  • Original language description

    Left ventricular hypertrophy may be a consequence of a hemodynamic overload or a manifestation of several diseases affecting different structural and functional proteins of cardiomyocytes. Among these, sarcomeric hypertrophic cardiomyopathy (HCM) represents the most frequent cause. In addition, several metabolic diseases lead to myocardial thickening, either due to intracellular storage (glycogen storage and lysosomal diseases), extracellular deposition (TTR and AL amyloidosis) or due to abnormal energy metabolism (mitochondrial diseases). The recognition of these rare causes of myocardial hypertrophy is important for family screening strategies, risk assessment, and treatment. Moreover, as there are specific therapies for some forms of HCM including enzyme substitution and chaperone therapies and specific treatments for TTR amyloidosis, a differential diagnosis should be sought in all patients with unexplained left ventricular hypertrophy. Diastolic dysfunction is a key feature of HCM and its phenocopies. Its assessment is complex and requires evaluation of several functional parameters and structural changes. Severe diastolic dysfunction carries a negative prognostic implication and its value in differential diagnosis is limited.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database

  • CEP classification

  • OECD FORD branch

    30201 - Cardiac and Cardiovascular systems

Result continuities

  • Project

  • Continuities

    V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Others

  • Publication year

    2018

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    International Journal of Cardiology

  • ISSN

    0167-5273

  • e-ISSN

  • Volume of the periodical

    257

  • Issue of the periodical within the volume

    April

  • Country of publishing house

    NL - THE KINGDOM OF THE NETHERLANDS

  • Number of pages

    7

  • Pages from-to

    344-350

  • UT code for WoS article

    000427530200088

  • EID of the result in the Scopus database

    2-s2.0-85042657256