International journal of cardiology · 2026
Dilated cardiomyopathy vs. non-dilated left ventricular dysfunction: genotype-phenotype relationship and outcome.
СтатьяНазвание и аннотация: Перевод в очереди
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Bellisario Irina, Aimo Alberto, Todiere Giancarlo, Alderotti Bianca, Gueli Ignazio Alessio, Grigoratos Chrysanthos, Ricci Fabrizio, De Gori Carmelo, Muca Matilda, Botto Nicoletta, Vittorini Simona, Gallina Sabina, Passino Claudio, Emdin Michele, Clemente Alberto, Barison Andrea
Аннотация
BACKGROUND: Dilated cardiomyopathy (DCM) and non-dilated left ventricular (LV) dysfunction (NDLVD) comprise patients with heterogeneous genetics and prognosis. This study aimed to investigate genotype-phenotype relationship and prognosis in DCM and NDLVD.
METHODS: Patients with a comprehensive clinical, genetic and CMR assessment, with LV ejection fraction (LVEF) <57% were included. Patients were categorized into DCM or NDLVD according to LV dilation (end-diastolic volume >96 ml/m2 in women, >105 ml/m2 in men). Patients with coronary artery disease, valvular, congenital, hypertrophic, inflammatory or infiltrative disease were excluded. The primary endpoint was a composite of cardiovascular death, sustained ventricular arrhythmias or appropriate defibrillator intervention. The secondary endpoint included also heart failure hospitalizations.
RESULTS: The cohort included 249 patients (age 53±14 years, 166 males) with median LVEF 45% (IQ range 33-51%), divided in 143 DCM and 106 NDLVD patients. Pathogenic/likely pathogenic mutations were present in 67 (27%), but were neither associated with phenotype, nor with prognosis. During a 72 (35-119) month follow-up, 54 patients (22%) experienced the composite event, 67 patients (27%) the secondary endpoint. At multivariate analysis, male sex, disease duration, natriuretic peptides, non-sustained ventricular arrhythmias, LVEF, left atrial volume and CMR-derived LV filling pressure predicted the primary endpoint (p<0.05). Moreover, disease duration, natriuretic peptides, non-sustained ventricular arrhythmias, right ventricular ejection fraction and CMR-derived LV filling pressure predicted the secondary endpoint (p<0.05).
CONCLUSIONS: In this cohort of patients with non-ischaemic cardiomyopathy, biventricular systolic function, left atrial volume and CMR-derived LV filling pressure were predictors of poor prognosis, while gene mutations were not.
METHODS: Patients with a comprehensive clinical, genetic and CMR assessment, with LV ejection fraction (LVEF) <57% were included. Patients were categorized into DCM or NDLVD according to LV dilation (end-diastolic volume >96 ml/m2 in women, >105 ml/m2 in men). Patients with coronary artery disease, valvular, congenital, hypertrophic, inflammatory or infiltrative disease were excluded. The primary endpoint was a composite of cardiovascular death, sustained ventricular arrhythmias or appropriate defibrillator intervention. The secondary endpoint included also heart failure hospitalizations.
RESULTS: The cohort included 249 patients (age 53±14 years, 166 males) with median LVEF 45% (IQ range 33-51%), divided in 143 DCM and 106 NDLVD patients. Pathogenic/likely pathogenic mutations were present in 67 (27%), but were neither associated with phenotype, nor with prognosis. During a 72 (35-119) month follow-up, 54 patients (22%) experienced the composite event, 67 patients (27%) the secondary endpoint. At multivariate analysis, male sex, disease duration, natriuretic peptides, non-sustained ventricular arrhythmias, LVEF, left atrial volume and CMR-derived LV filling pressure predicted the primary endpoint (p<0.05). Moreover, disease duration, natriuretic peptides, non-sustained ventricular arrhythmias, right ventricular ejection fraction and CMR-derived LV filling pressure predicted the secondary endpoint (p<0.05).
CONCLUSIONS: In this cohort of patients with non-ischaemic cardiomyopathy, biventricular systolic function, left atrial volume and CMR-derived LV filling pressure were predictors of poor prognosis, while gene mutations were not.