The new European Society of Cardiology guideline for the management of cardiomyopathies: key messages for cardiac electrophysiologists
AbstractElectrocardiographic findings and arrhythmias are common in cardiomyopathies. Both may be an early indication of a  specific diagnosis or may occur due to myocardial fibrosis and/or reduced contractility. Brady- and tachyarrhythmias significantly contribute to increased morbidity and mortality in patients with cardiomyopathies. Antiarrhythmic therapy including risk stratification is often challenging and plays a major role for these patients. Thus, an “electrophysiological” perspective on guidelines on cardiomyopathies may be warranted. As the European Society of Cardiology (ESC) has recently published a new...
Source: Herzschrittmachertherapie und Elektrophysiologie - November 16, 2023 Category: Cardiology Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Polish Heart Journal)
Source: Polish Heart Journal - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Kardiologia Polska)
Source: Kardiologia Polska - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Unveiling the structural and functional consequences of the p.D109G pathogenic mutation in human αB-Crystallin responsible for restrictive cardiomyopathy and skeletal myopathy
In this study, we generated this mutation in the α-Cry domain (ACD) which is crucial for forming the active chaperone dimeric state, using site-directed mutagenesis. After inducing expression in the bacterial host, we purified the mutant and wild-type recombinant proteins using anion exchange chromatography. Various spectroscopic evaluations revealed significant changes in the secondary, tertiary, and quaternary structures of human αB-Cry caused by this mutation. Furthermore, this pathogenic mutation led to the formation of protein oligomers with larger sizes than those of the wild-type protein counterpart. The mutant pr...
Source: International Journal of Biological Macromolecules - November 8, 2023 Category: Biochemistry Authors: Mehrnaz Hosseini Jafari Mohammad Bagher Shahsavani Masaru Hoshino Jun Hong Ali Akbar Saboury Ali Akbar Moosavi-Movahedi Reza Yousefi Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Polish Heart Journal)
Source: Polish Heart Journal - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Kardiologia Polska)
Source: Kardiologia Polska - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Unveiling the structural and functional consequences of the p.D109G pathogenic mutation in human αB-Crystallin responsible for restrictive cardiomyopathy and skeletal myopathy
In this study, we generated this mutation in the α-Cry domain (ACD) which is crucial for forming the active chaperone dimeric state, using site-directed mutagenesis. After inducing expression in the bacterial host, we purified the mutant and wild-type recombinant proteins using anion exchange chromatography. Various spectroscopic evaluations revealed significant changes in the secondary, tertiary, and quaternary structures of human αB-Cry caused by this mutation. Furthermore, this pathogenic mutation led to the formation of protein oligomers with larger sizes than those of the wild-type protein counterpart. The mutant pr...
Source: International Journal of Biological Macromolecules - November 8, 2023 Category: Biochemistry Authors: Mehrnaz Hosseini Jafari Mohammad Bagher Shahsavani Masaru Hoshino Jun Hong Ali Akbar Saboury Ali Akbar Moosavi-Movahedi Reza Yousefi Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Polish Heart Journal)
Source: Polish Heart Journal - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Kardiologia Polska)
Source: Kardiologia Polska - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Polish Heart Journal)
Source: Polish Heart Journal - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Kardiologia Polska)
Source: Kardiologia Polska - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Polish Heart Journal)
Source: Polish Heart Journal - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Kardiologia Polska)
Source: Kardiologia Polska - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research

Clinical features, etiology and survival in patients with restrictive cardiomyopathy: A single center experience
CONCLUSIONS: Genetic testing should be considered in every patient with RCM where light-chain amyloidosis has been excluded. Survival remains poor regardless of etiology. Increased concentrations of GDF15, hs-TNT, NT-proBNP and pericardial effusion are associated with worse prognosis. Further studies are warranted.PMID:37937352 | DOI:10.33963/v.kp.97879 (Source: Polish Heart Journal)
Source: Polish Heart Journal - November 8, 2023 Category: Cardiology Authors: Justyna A Szczygie ł Piotr Micha łek Gra żyna Truszkowska Joann Drozd-Soko łowska Aleksandra Wr óbel Maria Franaszczyk Monika Gawor-Prokopczyk Łukasz Mazurkiewicz Mateusz Ziarkiewicz Anna Waszczuk-Gajda Marta Legatowicz-Koprowska Ewa Walczak Piotr S Source Type: research