Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:70

Article

Singh, Rakesh K.^1,2  Canter, Charles E.³  Shi, Ling⁴  Colan, Steven D.⁵  Dodd, Debra A.^6,7  Everitt, Melanie D.^8,9  Hsu, Daphne T.¹⁰  Jefferies, John L.¹¹  Kantor, Paul F.¹²  Pahl, Elfriede¹³  Rossano, Joseph W.¹⁴  Towbin, Jeffrey A.¹⁵  Wilkinson, James D.^16,17  Lipshultz, Steven E.^16,17 

[1] Univ Calif San Diego, Dept Pediat, San Diego, CA 92103 USA

[2] Rady Childrens Hosp, San Diego, CA USA

[3] Washington Univ, Sch Med, Dept Pediat, St Louis, MO 63110 USA

[4] New England Res Inst, 9 Galen St, Watertown, MA 02172 USA

[5] Bostons Childrens Hosp, Dept Pediat, Boston, MA USA

[6] Vanderbilt Univ, Dept Pediat, Nashville, TN USA

[7] Monroe Carell Jr Childrens Hosp, Nashville, TN USA

[8] Childrens Hosp Colorado, Dept Pediat, Aurora, CO USA

[9] Univ Colorado, Aurora, CO USA

[10] Childrens Hosp Montefiore, Dept Pediat, Bronx, NY USA

[11] Cincinnati Childrens Hosp Med Ctr, Dept Pediat, Inst Heart, Cincinnati, OH 45229 USA

[12] Stollery Childrens Hosp, Dept Pediat, Edmonton, AB, Canada

[13] Ann & Robert H Lurie Childrens Hosp Chicago, Dept Pediat, Chicago, IL 60611 USA

[14] Childrens Hosp Philadelphia, Dept Pediat, Philadelphia, PA 19104 USA

[15] Le Bonheur Childrens Hosp, Dept Pediat, Memphis, TN USA

[16] Wayne State Univ, Sch Med, Dept Pediat, Detroit, MI 48201 USA

[17] Childrens Hosp Michigan, Detroit, MI 48201 USA

关键词: cardiomyopathy;    echocardiography;    heart failure;    pediatrics;    transplantation;   

DOI  :  10.1016/j.jacc.2017.09.1089

来源: Elsevier

【 摘 要 】

BACKGROUND Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation. OBJECTIVES This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death. METHODS Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort and to identify the factors associated with death. RESULTS Of 1,953 children, 1,199 were in the early cohort and 754 were in the late cohort. Most children in both cohorts had idiopathic DCM (64% vs. 63%, respectively). Median age (1.6 vs. 1.7 years), left ventricular end-diastolic z-scores (+4.2 vs. +4.2), and left ventricular fractional shortening (16% vs. 17%) at diagnosis were similar between cohorts. Although the rates of echocardiographic normalization (30% and 27%) and heart transplantation (24% and 24%) were similar, the death rate was higher in the early cohort than in the late cohort (18% vs. 9%; p = 0.04). Being in the early cohort (hazard ratio: 1.4; 95% confidence interval: 1.04 to 1.9; p = 0.03) independently predicted death. CONCLUSIONS Children with DCM have improved survival in the more recent era. This appears to be associated with factors other than heart transplantation, which was equally prevalent in both eras. (Pediatric Cardiomyopathy Registry [PCMR]; NCT00005391) (C) 2017 by the American College of Cardiology Foundation.

【 授权许可】

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