MOGE(S) Classification in Dilated Cardiomyopathy
What is the applicability and prognostic relevance of MOGE(S) (morphofunctional, organ involvement, genetic or familial, etiology, stage) classification in dilated cardiomyopathy (DCM)?
The study investigators used the Maastricht Cardiomyopathy Registry to identify patients with DCM. They excluded individuals with ischemic, hypertensive, valvular, and congenital heart disease. All the patients included in the study underwent complete diagnostic classification including genetic evaluation and endomyocardial biopsy (EMB). Inclusion criteria were as follows: 1) left ventricular ejection fraction (LVEF) <50% and indexed LV end-diastolic diameter >33 mm/m2 (men) or >32 mm/m2 (women); 2) EMB performed; 3) genetic evaluation, including counseling, pedigree analysis, and genetic testing in index patients; and 4) age ≥18 years. DCM was divided into seven causes, in line with the MOGE(S) classification: 1) genetic or familial; 2) virus-positive inflammatory (Vir+ Infl+); 3) virus-positive inflammatory-negative (Vir+ Infl−); 4) virus-negative inflammatory (Vir− Infl+); 5) virus-negative inflammatory-negative with proven systemic disease (Vir− Infl− systemic+); 6) rhythmogenic (tachycardiomyopathy not improving, >20% premature ventricular beats); and 7) toxic (alcohol abuse, hard drugs, chemotherapy).
The study cohort was comprised of 213 DCM patients, of which more than one-half of the patients were male. Genetic or familial DCM was diagnosed in approximately one-third of patients; 16% (n = 35) of the study cohort had organ involvement and 33% (n = 70) had genetic/familial DCM including 8% (n = 16) had a pathogenic mutation. In 73% of the patients (n = 155), at least one etiology was found, of whom 23% (n = 48) had more than one possible etiology. The cardiac presence of two viruses was seen in 23% (n = 47) of patients. Two patients had triple viral presence: parvovirus B19, human herpes virus-6, and Epstein-Barr virus. Viral presence was found in 79% (n = 47) of patients with earlier viral prodromes. Echocardiographic measurements after a mean of 1 year demonstrated LV reverse remodeling (LVRR) in 35% (n = 70) of patients. LVRR was more common in patients with nongenetic or nonfamilial DCM than in patients with genetic or familial DCM (40% vs. 25%; p = 0.04). The rate of LVRR was significantly higher in patients with toxic DCM versus not toxic DCM (75% vs. 33%; p = 0.003). The three toxic-triggered patients without LVRR after 12 months were persons who continued abusing alcohol. Whether patients had one cause or multiple possible causes, no significant differences in LVRR were seen after 12 months. After a median follow-up of 47 months, the presence of extracardiac organ involvement or New York Heart Association (NYHA) functional class ≥III showed a significantly worse outcome compared with those patients without extracardiac organ involvement or NYHA functional class
The authors concluded that MOGE(S) classification in DCM is applicable, and that each attribute and/or gene-environment interaction is a strong predictor of outcome.
The World Heart Federation published MOGE(S), a new classification scheme for cardiomyopathies, given the burgeoning knowledge in genetics of cardiomyopathy. This classification used a combination of phenotype, genetic variation, and etiology to classify patients. This study from Maastricht is important because it supports the relevance of this new classification and demonstrates that each attribute is an important predictor of outcome. The paradoxical finding that genetic or familial DCM was a negative predictor of short-term LVRR, whereas only the gene-environment interaction was a negative predictor of long-term outcome, was interesting. It would be interesting to see what data emerge when MOGE(S) classification is applied to other DCM cohorts.
Keywords: Alcoholism, Biopsy, Cardiomyopathies, Cardiomyopathy, Dilated, Echocardiography, Heart Failure, Gene-Environment Interaction, Genetic Testing, Genetic Variation, Parvovirus, Stroke Volume, Ventricular Premature Complexes
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