Tropomyosin-troponin complex in inherited cardiomyopathies
The tropomyosin-troponin complex (TTC) regulates cardiac contractility by controlling cross-bridge attachments to actin filaments based on intracellular Ca2+ levels. TTC consists of tropomyosin (TPM), cardiac troponin-T (cTnT), cardiac troponin-I (cTnI), and cardiac troponin-C (cTnC). Pathogenic variants (mutations) in genes encoding these proteins (TPM1 [TPM], TNNT2 [cTnT], TNNI3 [cTnI], and TNNC1 [cTnC]) cause different cardiomyopathy types, including hypertrophic (HCM), dilated (DCM), or restrictive cardiomyopathy (RCM). (Source: Heart Rhythm)
Source: Heart Rhythm - February 19, 2024 Category: Cardiology Authors: Christian Krijger Ju árez, Vasco Sequeira, Malou van den Boogaard, Christiaan C. Veerman, Nicola J. Hoetjes, Edwin Poel, Michael W.T. Tanck, Ronald H. Lekanne Deprez, Alexa M.C. Vermeer, Jolanda van der Velden, Ahmad S. Amin Source Type: research

Tropomyosin –troponin complex in inherited cardiomyopathies
The tropomyosin –troponin complex (TTC) regulates cardiac contractility by controlling cross-bridge attachments to actin filaments based on intracellular Ca2+ levels. TTC consists of tropomyosin (TPM), cardiac troponin-T (cTnT), cardiac troponin-I (cTnI), and cardiac troponin-C (cTnC). Pathogenic variants (mutati ons) in genes encoding these proteins (TPM1 [TPM], TNNT2 [cTnT], TNNI3 [cTnI], and TNNC1 [cTnC]) cause different cardiomyopathy types, including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), and restrictive cardiomyopathy (RCM). (Source: Heart Rhythm)
Source: Heart Rhythm - February 19, 2024 Category: Cardiology Authors: Christian Krijger Ju árez, Vasco Sequeira, Malou van den Boogaard, Christiaan C. Veerman, Nicola J. Hoetjes, Edwin Poel, Michael W.T. Tanck, Ronald H. Lekanne Deprez, Alexa M.C. Vermeer, Jolanda van der Velden, Ahmad S. Amin Tags: Research Letter Source Type: research

The bird hunting story: constrictive pericarditis and restrictive cardiomyopathy
(Source: Indian Journal of Thoracic and Cardiovascular Surgery)
Source: Indian Journal of Thoracic and Cardiovascular Surgery - February 6, 2024 Category: Cardiovascular & Thoracic Surgery Source Type: research

The clinical profile, genetic basis and survival of childhood cardiomyopathy: a single-center retrospective study
Conclusions: This study demonstrates that CM, especially RCM, is related to a high incidence of death. NYHA/Ross class III or IV is a predictor of mortality in the patients and gene mutations may be a common cause.Trial registration: MR-50-23-011798.What is Known:• Cardiomyopathy (CM) is a heterogeneous group of myocardial diseases and one of the leading causes of heart failure in children due to the lack of effective treatments.• There remains scarce data on Asian pediatric populations though emerging studies have assessed the clinical characteristics and outcomes of CM.What is New:• A retrospective study was conduc...
Source: European Journal of Pediatrics - January 2, 2024 Category: Pediatrics Source Type: research

Assessing Screening For Transthyretin Cardiac Amyloidosis Among Patients At A Safety Net Hospital In Metro Atlanta
Cardiac amyloidosis is a rare and progressive form of restrictive cardiomyopathy which is associated with a poor prognosis. Two types of amyloid proteins commonly infiltrate the heart: i) immunoglobulin light-chain (AL or primary systemic) amyloid and ii) transthyretin (ATTR) amyloid. ATTR is the predominant hereditary form of cardiac amyloidosis, characterized by abnormal deposition of insoluble misfolded transthyretin protein fibrils in the myocardium resulting in organ dysfunction. The actual prevalence of ATTR amyloidosis is unknown, although recent data suggest that it is overlooked as a cause of common cardiovascular...
Source: Journal of Cardiac Failure - January 1, 2024 Category: Cardiology Authors: Christabel E. Nyange, Valery Effoe, Jo Ann Cross, Samed Obeng, Hassan Abbas, Judith Volcy, Anekwe Onwuanyi Tags: 446 Source Type: research

Application of Routine Echocardiography Combined with Contrast-Enhanced Echocardiography in the Diagnosis of Endomyocardial Fibrosis
CONCLUSIONS: EMF has characteristic RE presentation, and different EMF subtypes have unique characteristics. CEE can better display heart structure, ventricular wall motion, and tissue perfusion compared to RE. A combination of RE and CEE can make the EMF diagnosis more accurate, thereby allowing early treatment for EMF patients.PMID:38058070 | DOI:10.24976/Discov.Med.202335179.101 (Source: Discovery Medicine)
Source: Discovery Medicine - December 7, 2023 Category: Research Authors: Bijun Wu Yaolin Yang Ping Zhao Source Type: research