Author
T. M. Yelbuz
Bio: T. M. Yelbuz is an academic researcher. The author has contributed to research in topics: Cardiomyopathy. The author has an hindex of 1, co-authored 1 publications receiving 273 citations.
Topics: Cardiomyopathy
Papers
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TL;DR: Echocardiography has been shown to be the method of choice in diagnosis of INVM and the establishment of a registry, which was initiated by the "Arbeitsgemeinschaft Leitende Kardiologische Krankenhausärzte (ALKK)," may provide further clues for diagnosis, risk stratification, and management of this disease.
Abstract: Isolated noncompaction of the left ventricular myocardium (INVM), first described in 1984, is an unclassified cardiomyopathy and is assumed to occur as an arrest of the compaction process during the normal development of the heart. Between weeks 5 to 8 of human fetal development, the ventricular myocardium undergoes gradual compaction with transformation of the relatively large intertrabecular spaces into capillaries while the residual spaces within the trabecular meshwork gradually flatten or disappear. In the case of INVM, the spaces within the intertrabecular meshwork persist while no other cardiac abnormalities exist. Although there is substantial evidence supporting the developmental hypothesis, other pathogenetic processes responsible for INVM have been discussed. It can be assumed that INVM will be better understood in the future as the molecular genetic basis of cardiomyopathies will be further unravelled. Echocardiography has been shown to be the method of choice in diagnosis of INVM. The diagnostic criteria can be summarized as: 1) appearance of at least four prominent trabeculations and deep intertrabecular recesses; 2) appearance of blood flow from the ventricular cavity into the intertrabecular recesses as visualized by color Doppler imaging; 3) the segments of noncompacted myocardium mainly involve the apex and the inferior mid and lateral mid of the left ventricular wall and typically show a two-layered structure with an endsystolic ratio greater than two between the noncompacted subendocardial layer and the compacted subepicardial layer; 4) absence of coexisting cardiac abnormalities. Magnetic resonance imaging using modern gradient echo sequences has also been shown to diagnose INVM accurately. The clinical presentation of INVM is characterized by a high prevalence of heart failure, thromboembolic events and arrhythmias including ventricular tachycardia and atrial fibrillation. The establishment of a registry, which was initiated by the "Arbeitsgemeinschaft Leitende Kardiologische Krankenhausarzte (ALKK)" recently, may provide further clues for diagnosis, risk stratification, and management of this disease.
299 citations
Cited by
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Mayo Clinic1, New York University2, University of Hamburg3, Children's National Medical Center4, University of Girona5, Johns Hopkins University6, St George's, University of London7, Harvard University8, University of Ottawa9, University of Toronto10, University of Miami11, University of Paris12, University College London13, University of Münster14, University of Sydney15, Cincinnati Children's Hospital Medical Center16, University of Oxford17, University of Amsterdam18, Indiana University19
TL;DR: This dissertation aims to provide a history of modern medicine and some of the techniques and practices used in modern medicine, as well as some new approaches, that were introduced in the field of medicine more than 40 years ago.
1,254 citations
Mayo Clinic1, New York University2, University of Hamburg3, George Washington University4, University of Girona5, Johns Hopkins University6, St George's, University of London7, Harvard University8, University of Ottawa9, Hospital for Sick Children10, University of Miami11, Paris Descartes University12, University College London13, University of Sydney14, Cincinnati Children's Hospital Medical Center15, John Radcliffe Hospital16, University of Amsterdam17, Indiana University18
TL;DR: This international consensus statement provides the state of genetic testing for the channelopathy and cardiomyopathies and summarizes the opinion of the international writing group members based on their own experience and on a general review of the literature with respect to the use and role of geneticTesting for these potentially heritable cardiac conditions.
Abstract: This international consensus statement provides the state of genetic testing for the channelopathies and cardiomyopathies. It summarizes the opinion of the international writing group members based on their own experience and on a general review of the literature with respect to the use and role of genetic testing for these potentially heritable cardiac conditions. This document focuses primarily on the state of genetic testing for the 13 distinct entities detailed and the relative diagnostic, prognostic, and therapeutic impact of the genetic test result for each entity. It does not focus on the therapeutic management of the various channelopathies and cardiomyopathies. Treatment/management issues are only discussed for those diseases (i.e., LQTS, HCM, DCM + CCD, RCM) in which the genetic test result could potentially influence treatment considerations.
Writing recommendations for genetic diseases require adaptation of the methodology normally adopted to prepare guidelines for clinical practice. Documents produced by other scientific societies have acknowledged the need to define the criteria used to rank the strength of recommendation for genetic diseases.1
The most obvious difference is that randomized and/or blinded studies do not exist. Instead, most of the available data are derived from registries that have followed patients and recorded outcome information. The authors of this statement have therefore defined specific criteria for Class I, Class IIa or b, and Class III recommendations and have used the conventional language adopted by AHA/ACC/ESC Guidelines to express each class. All recommendations are level of evidence (LOE) C (i.e., based on experts' opinions).
A Class I recommendation ( “is recommended” ) was applied for genetic testing in index cases with a sound clinical suspicion for the presence of a channelopathy or a cardiomyopathy when the positive predictive value of a genetic test is high (likelihood of positive result >40% and signal/noise ratio >10; Table 3), AND/OR when …
1,147 citations
TL;DR: The American College of Cardiology Foundation Board of Trustees in February 2008, document be cited as follows.
793 citations
University of Tennessee Health Science Center1, University College London2, Harvard University3, Mayo Clinic4, Johns Hopkins University5, University of São Paulo6, Duke University7, University of California, Los Angeles8, Cleveland Clinic9, University of Pittsburgh10, Peking Union Medical College11, University of Arizona12, University of Sydney13, Vanderbilt University14, Medical University of South Carolina15, University of British Columbia16, University of Texas Southwestern Medical Center17, Anschutz Medical Campus18, University of Pavia19, Nippon Medical School20, University of Amsterdam21, Utrecht University22, Columbia University23, University of Rochester24
TL;DR: This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms.
439 citations
TL;DR: Treating of arrhythmia and implantation of an automatic implantable cardioverter-defibrillator for prevention of sudden death are mainstays of therapy when deemed necessary and appropriate in patients with systolic dysfunction.
343 citations