TY - JOUR
T1 - Prognosis and clinical characteristics of dilated cardiomyopathy with family history via pedigree analysis
AU - Marume, Kyohei
AU - Noguchi, Teruo
AU - Tateishi, Emi
AU - Morita, Yoshiaki
AU - Miura, Hiroyuki
AU - Nishimura, Kunihiro
AU - Ohta-Ogo, Keiko
AU - Yamada, Naoaki
AU - Tsujita, Kenichi
AU - Izumi, Chisato
AU - Kusano, Kengo
AU - Ogawa, Hisao
AU - Yasuda, Satoshi
N1 - Funding Information:
The present work was supported in part by the Takeda Science Foundation and the Japan Cardiovascular Research Foundation. H.O. and S.Y. are members of Circulation Journal’ Editorial Team.
Publisher Copyright:
© 2020 Japanese Circulation Society. All rights reserved.
PY - 2020
Y1 - 2020
N2 - Background: The clinical characteristics and prognostic outcomes of dilated cardiomyopathy (DCM) with a familial history (FHx) via pedigree analysis are unclear. Methods and Results: We conducted a prospective observational study of 514 consecutive Japanese patients with DCM. FHx was defined as the presence of DCM in ≥1 family member within 2-degrees relative based on pedigree analysis. The primary endpoint was a composite of major cardiac events (sudden cardiac death and pump failure death). The prevalence of FHx was 7.4% (n=38). During a median follow-up of 3.6 years, 77 (15%) patients experienced a major cardiac event. Multivariable Cox regression analysis identified FHx as independently associated with major cardiac events (hazard ratio [HR] 4.32; 95% confidence interval [CI], 2.04–9.19; P<0.001) compared with conventional risk factors such as age, QRS duration, and left ventricular volume. In the propensity score-matched cohort (n=38 each), the FHx group had a significantly higher incidence of major cardiac events (HR, 4.48; 95% CI, 1.25–16.13; P=0.022). In addition, the FHx group had a higher prevalence of a diffuse late gadolinium enhancement (LGE) pattern than the no-FHx group (32% vs. 17%, P=0.022). Conclusions: DCM patients with FHx had a worse prognosis, which was associated with a higher prevalence of a diffuse LGE pattern, than patients without FHx.
AB - Background: The clinical characteristics and prognostic outcomes of dilated cardiomyopathy (DCM) with a familial history (FHx) via pedigree analysis are unclear. Methods and Results: We conducted a prospective observational study of 514 consecutive Japanese patients with DCM. FHx was defined as the presence of DCM in ≥1 family member within 2-degrees relative based on pedigree analysis. The primary endpoint was a composite of major cardiac events (sudden cardiac death and pump failure death). The prevalence of FHx was 7.4% (n=38). During a median follow-up of 3.6 years, 77 (15%) patients experienced a major cardiac event. Multivariable Cox regression analysis identified FHx as independently associated with major cardiac events (hazard ratio [HR] 4.32; 95% confidence interval [CI], 2.04–9.19; P<0.001) compared with conventional risk factors such as age, QRS duration, and left ventricular volume. In the propensity score-matched cohort (n=38 each), the FHx group had a significantly higher incidence of major cardiac events (HR, 4.48; 95% CI, 1.25–16.13; P=0.022). In addition, the FHx group had a higher prevalence of a diffuse late gadolinium enhancement (LGE) pattern than the no-FHx group (32% vs. 17%, P=0.022). Conclusions: DCM patients with FHx had a worse prognosis, which was associated with a higher prevalence of a diffuse LGE pattern, than patients without FHx.
KW - Cardiac events
KW - Dilated cardiomyopathy
KW - Familial history
KW - Late gadolinium enhancement
KW - Magnetic resonance
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U2 - 10.1253/circj.CJ-19-1176
DO - 10.1253/circj.CJ-19-1176
M3 - Article
C2 - 32624524
AN - SCOPUS:85088678867
SN - 1346-9843
VL - 84
SP - 1284
EP - 1293
JO - Circulation Journal
JF - Circulation Journal
IS - 8
ER -