TY - JOUR
T1 - Austrian consensus statement on the diagnosis and management of hypertrophic cardiomyopathy
AU - Verheyen, Nicolas
AU - Auer, Johannes
AU - Bonaros, Nikolaos
AU - Buchacher, Tamara
AU - Dalos, Daniel
AU - Grimm, Michael
AU - Mayr, Agnes
AU - Rab, Anna
AU - Reinstadler, Sebastian
AU - Scherr, Daniel
AU - Toth, Gabor G.
AU - Weber, Thomas
AU - Zach, David K.
AU - Zaruba, Marc-Michael
AU - Zimpfer, Daniel
AU - Rainer, Peter P.
AU - Poelzl, Gerhard
N1 - Lehr-KH Kardinal Schwarzenberg Klinikum, Schwarzach, Austria und St. Josef Hospital Braunau, Braunau, Austria Paracelsus Medical University Salzburg, Salzburg, Austria
PY - 2024/10
Y1 - 2024/10
N2 - Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease that is characterized by left ventricular hypertrophy unexplained by secondary causes. Based on international epidemiological data, around 20,000-40,000 patients are expected to be affected in Austria. Due to the wide variety of clinical and morphological manifestations the diagnosis can be difficult and the disease therefore often goes unrecognized. HCM is associated with a substantial reduction in quality of life and can lead to sudden cardiac death, especially in younger patients. Early and correct diagnosis, including genetic testing, is essential for comprehensive counselling of patients and their families and for effective treatment. The latter is especially true as an effective treatment of outflow tract obstruction has recently become available in the form of a first in class cardiac myosin ATPase inhibitor, as a noninvasive alternative to established septal reduction therapies. The aim of this Austrian consensus statement is to summarize the recommendations of international guidelines with respect to the genetic background, pathophysiology, diagnostics and management in the context of the Austrian healthcare system and resources, and to present them in easy to understand algorithms.
AB - Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease that is characterized by left ventricular hypertrophy unexplained by secondary causes. Based on international epidemiological data, around 20,000-40,000 patients are expected to be affected in Austria. Due to the wide variety of clinical and morphological manifestations the diagnosis can be difficult and the disease therefore often goes unrecognized. HCM is associated with a substantial reduction in quality of life and can lead to sudden cardiac death, especially in younger patients. Early and correct diagnosis, including genetic testing, is essential for comprehensive counselling of patients and their families and for effective treatment. The latter is especially true as an effective treatment of outflow tract obstruction has recently become available in the form of a first in class cardiac myosin ATPase inhibitor, as a noninvasive alternative to established septal reduction therapies. The aim of this Austrian consensus statement is to summarize the recommendations of international guidelines with respect to the genetic background, pathophysiology, diagnostics and management in the context of the Austrian healthcare system and resources, and to present them in easy to understand algorithms.
KW - Heart failure
KW - Hypertrophic phenotype
KW - Left ventricular outflow tract obstruction
KW - Phenocopies
KW - Sudden cardiac death
UR - https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=pmu_pure&SrcAuth=WosAPI&KeyUT=WOS:001324134200001&DestLinkType=FullRecord&DestApp=WOS_CPL
U2 - 10.1007/s00508-024-02442-1
DO - 10.1007/s00508-024-02442-1
M3 - Original Article
C2 - 39352517
SN - 0043-5325
VL - 136
SP - 571
EP - 597
JO - WIENER KLINISCHE WOCHENSCHRIFT
JF - WIENER KLINISCHE WOCHENSCHRIFT
IS - SUPPL 15
ER -