title: The MOGE(S) classification of cardiomyopathy for clinicians.
abstract: Most cardiomyopathies are familial diseases. Cascade family screening identifies asymptomatic patients and family members with early traits of disease. The inheritance is autosomal dominant in a majority of cases, and recessive, X-linked, or matrilinear in the remaining. For the last 50 years, cardiomyopathy classifications have been based on the morphofunctional phenotypes, allowing cardiologists to conveniently group them in broad descriptive categories. However, the phenotype may not always conform to the genetic characteristics, may not allow risk stratification, and may not provide pre-clinical diagnoses in the family members. Because genetic testing is now increasingly becoming a part of clinical work-up, and based on the genetic heterogeneity, numerous new names are being coined for the description of cardiomyopathies associated with mutations in different genes; a comprehensive nosology is needed that could inform the clinical phenotype and involvement of organs other than the heart, as well as the genotype and the mode of inheritance. The recently proposed MOGE(S) nosology system embodies all of these characteristics, and describes the morphofunctional phenotype (M), organ(s) involvement (O), genetic inheritance pattern (G), etiological annotation (E) including genetic defect or underlying disease/substrate, and the functional status (S) of the disease using both the American College of Cardiology/American Heart Association stage and New York Heart Association functional class. The proposed nomenclature is supported by a web-assisted application and assists in the description of cardiomyopathy in symptomatic or asymptomatic patients and family members in the context of genetic testing. It is expected that such a nomenclature would help group cardiomyopathies on their etiological basis, describe complex genetics, and create collaborative registries.
authors.affiliation: Center for Inherited Cardiovascular Diseases, IRCCS Foundation Policlinico San Matteo, Pavia, Italy.
authors.forename: Eloisa
authors.initials: E
authors.lastname: Arbustini
authors.affiliation: Weill Cornell Medical College, New York, New York.
authors.forename: Navneet
authors.initials: N
authors.lastname: Narula
authors.affiliation: GVM Care & Research, E.S. Health Science Foundation, Maria Cecilia Hospital, Cotignola, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Tavazzi
authors.affiliation: Center for Inherited Cardiovascular Diseases, IRCCS Foundation Policlinico San Matteo, Pavia, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Serio
authors.affiliation: Center for Inherited Cardiovascular Diseases, IRCCS Foundation Policlinico San Matteo, Pavia, Italy.
authors.forename: Maurizia
authors.initials: M
authors.lastname: Grasso
authors.affiliation: Center for Inherited Cardiovascular Diseases, IRCCS Foundation Policlinico San Matteo, Pavia, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Favalli
authors.affiliation: University of Pavia, Pavia, Italy.
authors.forename: Riccardo
authors.initials: R
authors.lastname: Bellazzi
authors.affiliation: St. Luke's Medical Center, Milwaukee, Wisconsin.
authors.forename: Jamil A
authors.initials: JA
authors.lastname: Tajik
authors.forename: Robert O
authors.initials: RO
authors.lastname: Bonow
authors.affiliation: Northwestern University School of Medicine, Chicago, Illinois.
authors.forename: Robert D
authors.initials: RD
authors.lastname: Bonow
authors.affiliation: Icahn School of Medicine at Mount Sinai, New York, New York.
authors.forename: Valentin
authors.initials: V
authors.lastname: Fuster
authors.affiliation: Icahn School of Medicine at Mount Sinai, New York, New York. Electronic address: Narula@mountsinai.org.
authors.forename: Jagat
authors.initials: J
authors.lastname: Narula
mesh_terms: D002309:Cardiology / Q000379:methods* / Q000639:trends; D009202:Cardiomyopathies / Q000145:classification* / Q000175:diagnosis / Q000235:genetics*; D020022:Genetic Predisposition to Disease / Q000145:classification / Q000235:genetics; D005820:Genetic Testing / Q000379:methods; D006801:Humans; D010820:Physicians
keywords: cardiomyopathy; classification; genetics