Recovery of Echocardiographic Function in Children With Idiopathic Dilated Cardiomyopathy

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:63

Article

Everitt, Melanie D.¹  Sleeper, Lynn A.²  Lu, Minmin²  Canter, Charles E.³  Pahl, Elfriede⁴  Wilkinson, James D.⁵  Addonizio, Linda J.⁶  Towbin, Jeffrey A.⁷  Rossano, Joseph⁸  Singh, Rakesh K.⁹  Lamour, Jacqueline⁹  Webber, Steven A.¹⁰  Colan, Steven D.¹¹  Margossian, Renee¹¹  Kantor, Paul F.¹²  Jefferies, John L.⁹  Lipshultz, Steven E.⁶ 

[1] Primary Childrens Med Ctr, Salt Lake City, UT 84103 USA

[2] New England Res Inst Inc, Watertown, MA USA

[3] Washington Univ, St Louis, MO USA

[4] Ann & Robert H Lurie Childrens Hosp, Chicago, IL USA

[5] Univ Miami, Miller Sch Med, Dept Pediat, Miami, FL 33136 USA

[6] Columbia Univ, New York, NY USA

[7] Cincinnati Childrens Hosp Med Ctr, Inst Heart, Cincinnati, OH 45229 USA

[8] Univ Penn, Philadelphia, PA 19104 USA

[9] Childrens Hosp Montefiore, Bronx, NY USA

[10] Vanderbilt Univ, Nashville, TN 37235 USA

[11] Boston Childrens Hosp, Boston, MA USA

[12] Univ Alberta, Stollery Childrens Hosp, Edmonton, AB T6G 2M7, Canada

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

DOI  :  10.1016/j.jacc.2013.11.059

来源: Elsevier

【 摘 要 】

Objectives This study sought to determine the incidence and predictors of recovery of normal echocardiographic function among children with idiopathic dilated cardiomyopathy (DCM). Background Most children with idiopathic DCM have poor outcomes; however, some improve. Methods We studied children <18 years of age from the Pediatric Cardiomyopathy Registry who had both depressed left ventricular (LV) function (fractional shortening or ejection fraction z-score <- 2) and LV dilation (end-diastolic dimension [LVEDD] z-score > 2) at diagnosis and who had at least 1 follow-up echocardiogram 30 days to 2 years from the initial echocardiogram. We estimated the cumulative incidence and predictors of normalization. Results Among 868 children who met the inclusion criteria, 741 (85%) had both echocardiograms. At 2 years, 22% had recovered normal LV function and size; 51% had died or undergone heart transplantation (median, 3.2 months), and 27% had persistently abnormal echocardiograms. Younger age (hazard ratio [HR]: 0.92; 95% confidence interval [CI]: 0.88 to 0.97) and lower LVEDD z-score (HR: 0.78; 95% CI: 0.70 to 0.87) independently predicted normalization. Nine children (9%) with normal LV function and size within 2 years of diagnosis later underwent heart transplantation or died. Conclusions Despite marked LV dilation and depressed function initially, children with idiopathic DCM can recover normal LV size and function, particularly those younger and with less LV dilation at diagnosis. Investigations related to predictors of recovery, such as genetic associations, serum markers, and the impact of medical therapy or ventricular unloading with assist devices are important next steps. Longer follow- up after normalization is warranted as cardiac failure can recur. (Pediatric Cardiomyopathy Registry; NCT00005391) (c) 2014 by the American College of Cardiology Foundation

【 授权许可】

Free