Prognosis and Clinical Characteristics of Dilated Cardiomyopathy With Family History via Pedigree Analysis

被引:8
作者
Marume, Kyohei [1 ,5 ]
Noguchi, Teruo [1 ]
Tateishi, Emi [2 ]
Morita, Yoshiaki [2 ,6 ]
Miura, Hiroyuki [1 ]
Nishimura, Kunihiro [3 ]
Ohta-Ogo, Keiko [4 ]
Yamada, Naoaki [7 ]
Tsujita, Kenichi [5 ]
Izumi, Chisato [1 ]
Kusano, Kengo [1 ]
Ogawa, Hisao [1 ]
Yasuda, Satoshi [1 ]
机构
[1] Natl Cerebral & Cardiovasc Ctr, Dept Cardiovasc Med, 6-1 Kishibe Shinmachi, Suita, Osaka 5648565, Japan
[2] Natl Cerebral & Cardiovasc Ctr, Dept Radiol, Suita, Osaka, Japan
[3] Natl Cerebral & Cardiovasc Ctr, Dept Stat & Data Anal, Suita, Osaka, Japan
[4] Natl Cerebral & Cardiovasc Ctr, Dept Pathol, Suita, Osaka, Japan
[5] Kumamoto Univ, Grad Sch Med Sci, Dept Cardiovasc Med, Kumamoto, Japan
[6] Tohoku Univ Hosp, Dept Radiol, Sendai, Miyagi, Japan
[7] Osaka Neurol Inst, Dept Radiol, Toyonaka, Osaka, Japan
关键词
Cardiac events; Dilated cardiomyopathy; Familial history; Late gadolinium enhancement; Magnetic resonance; ASSOCIATION-TASK-FORCE; KEY DATA ELEMENTS; WRITING COMMITTEE; AMERICAN-COLLEGE; DATA STANDARDS; FREQUENCY; DEFINITIONS; MORTALITY; GENETICS; FIBROSIS;
D O I
10.1253/circj.CJ-19-1176
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:1284 / +
页数:14
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