PMID- 32624524 OWN - NLM STAT- MEDLINE DCOM- 20201207 LR - 20201214 IS - 1347-4820 (Electronic) IS - 1346-9843 (Linking) VI - 84 IP - 8 DP - 2020 Jul 22 TI - Prognosis and Clinical Characteristics of Dilated Cardiomyopathy With Family History via Pedigree Analysis. PG - 1284-1293 LID - 10.1253/circj.CJ-19-1176 [doi] 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. FAU - Marume, Kyohei AU - Marume K AD - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center. AD - Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University. FAU - Noguchi, Teruo AU - Noguchi T AD - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center. FAU - Tateishi, Emi AU - Tateishi E AD - Department of Radiology, National Cerebral and Cardiovascular Center. FAU - Morita, Yoshiaki AU - Morita Y AD - Department of Radiology, National Cerebral and Cardiovascular Center. AD - Department of Radiology, Tohoku University Hospital. FAU - Miura, Hiroyuki AU - Miura H AD - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center. FAU - Nishimura, Kunihiro AU - Nishimura K AD - Department of Statistics and Data Analysis, National Cerebral and Cardiovascular Center. FAU - Ohta-Ogo, Keiko AU - Ohta-Ogo K AD - Department of Pathology, National Cerebral and Cardiovascular Center. FAU - Yamada, Naoaki AU - Yamada N AD - Department of Radiology, Osaka Neurological Institute. FAU - Tsujita, Kenichi AU - Tsujita K AD - Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University. FAU - Izumi, Chisato AU - Izumi C AD - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center. FAU - Kusano, Kengo AU - Kusano K AD - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center. FAU - Ogawa, Hisao AU - Ogawa H AD - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center. FAU - Yasuda, Satoshi AU - Yasuda S AD - Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center. LA - eng PT - Comparative Study PT - Journal Article PT - Observational Study DEP - 20200703 PL - Japan TA - Circ J JT - Circulation journal : official journal of the Japanese Circulation Society JID - 101137683 SB - IM MH - Adult MH - Aged MH - Cardiomyopathy, Dilated/diagnostic imaging/*genetics/mortality/physiopathology MH - Disease Progression MH - Female MH - Fibrosis MH - Genetic Predisposition to Disease MH - Heart Disease Risk Factors MH - *Heredity MH - Humans MH - Incidence MH - Japan/epidemiology MH - Magnetic Resonance Imaging MH - Male MH - Middle Aged MH - Myocardium/pathology MH - *Pedigree MH - Phenotype MH - Prevalence MH - Prognosis MH - Prospective Studies MH - Ventricular Remodeling OTO - NOTNLM OT - Cardiac events OT - Dilated cardiomyopathy OT - Familial history OT - Late gadolinium enhancement OT - Magnetic resonance EDAT- 2020/07/07 06:00 MHDA- 2020/12/15 06:00 CRDT- 2020/07/07 06:00 PHST- 2020/07/07 06:00 [pubmed] PHST- 2020/12/15 06:00 [medline] PHST- 2020/07/07 06:00 [entrez] AID - 10.1253/circj.CJ-19-1176 [doi] PST - ppublish SO - Circ J. 2020 Jul 22;84(8):1284-1293. doi: 10.1253/circj.CJ-19-1176. Epub 2020 Jul 3.