Abstract
New approaches to pediatric dilated cardiomyopathy in current practice
Highlights
The importance of myocardic pathology as a cause of pediatric death of cardiac origin cannot be forseen
Cardiomyopathies are defined as anomalies with initial structural and functional involvement of the myocardium, after excluding conditions which evolve to severe myocardial dysfunction in the absence of proper treatment [1]
Starting with 2013 a new, much more elaborated classification system has been implemented, known under the MOGE (S) aconym, a descriptive system in which informations provided by molecular genetic testings created the premises towards a better knowledge of physiopathogical basis, implementation of a descriptive nosologic phenotype-genotype and establisment of an accurate clinic and genetic diagnosis [3,4]
Summary
The importance of myocardic pathology as a cause of pediatric death of cardiac origin cannot be forseen. Cardiomyopathies are defined as anomalies with initial structural and functional involvement of the myocardium, after excluding conditions which evolve to severe myocardial dysfunction in the absence of proper treatment (serious cardiac congenital anomalies, arterial hypertension) [1]. Starting with 2013 a new, much more elaborated classification system has been implemented, known under the MOGE (S) aconym, a descriptive system in which informations provided by molecular genetic testings created the premises towards a better knowledge of physiopathogical basis, implementation of a descriptive nosologic phenotype-genotype and establisment of an accurate clinic and genetic diagnosis [3,4]. A detailed, descriptive diagnosis is concluded for each patient, depending on their specific clinic and genetic characteristics (phenotype/genotype) and the management of cardiomyopathies becomes personalised (target of future medicine) [5]. Test establishes a prediction index and enhances the development of a follow-up strategy regarding letal arrhythmia risk in case of „malignant” mutations or sudden death risk, as well as prediction of disease transmission to the succesors) [6]
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