Twenty years of progress and beckoning frontiers in cardiovascular pathology: cardiomyopathies.
In the last 20 years, with the advent of cardiac transplantation and the availability of molecular biology techniques, major advancements were achieved in the understanding of cardiomyopathies. Novel cardiomyopathies have been discovered (arrhythmogenic right ventricular, primary restrictive, and noncompacted myocardium) and added in the update of WHO classification. Myocarditis was also included with the name "inflammatory cardiomyopathy." Adenoviruses and parvoviruses were found to be frequent cardiotropic viruses in addition to enteroviruses. The extraordinary progress accomplished in molecular genetics of inherited cardiomyopathies allowed to establish hypertrophic and restrictive cardiomyopathies as sarcomeric ("force generation") diseases, dilated cardiomyopathies as cytoskeleton ("force transmission") disease, and arrhythmogenic right ventricular cardiomyopathy (ARVC) as cell junction disease. If we consider also cardiomyopathy as ion channel disease (long and short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), because they are diseases of the myocardium associated with electrical dysfunction, then a genomic/postgenomic classification of inherited cardiomyopathies may be put forward: cytoskeletal cardiomyopathy, sarcomeric cardiomyopathy, cell junction cardiomyopathy and ion channel cardiomyopathy.
Thiene
Gaetano
G
Institute of Pathological Anatomy, University of Padua Medical School, Padua, Italy. agetano.thiene@unipd.it
Basso
Cristina
C
Calabrese
Fiorella
F
Angelini
Annalisa
A
Valente
Marialuisa
M
D000258:Adenovirus Infections, Human / Q000150:complications; D000818:Animals; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000145:classification / Q000235:genetics / Q000473:pathology; D002309:Cardiology / Q000639:trends*; D009202:Cardiomyopathies / Q000145:classification* / Q000235:genetics / Q000821:virology; D002313:Cardiomyopathy, Restrictive / Q000145:classification / Q000473:pathology; D006801:Humans; D009205:Myocarditis / Q000145:classification / Q000503:physiopathology; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D010322:Parvoviridae Infections / Q000150:complications; D014944:World Health Organization