- 作者：Melanie D. Everitt ; MD&lowast ; ; Melanie.everitt@imail.org" class="auth_mail ; Lynn A. Sleeper ; ScD&dagger ; ; Minmin Lu ; MS&dagger ; ; Charles E. Canter ; MD&Dagger ; ; Elfriede Pahl ; MD§ ; ; James D. Wilkinson ; MD ; MPH鈥?/sup> ; Linda J. Addonizio ; MD¶ ; ; Jeffrey A. Towbin ; MD# ; Joseph Rossano ; MD&lowast ; &lowast ; ; Rakesh K. Singh ; MD ; MS&dagger ; &dagger ; ; Jacqueline Lamour ; MD&dagger ; &dagger ; ; Steven A. Webber ; MBChB&Dagger ; &Dagger ; ; Steven D. Colan ; MD§ ; § ; ; Renee Margossian ; MD§ ; § ; ; Paul F. Kantor ; MBBCh鈥栤€?/sup> ; John L. Jefferies ; MD&dagger ; &dagger ; ; Steven E. Lipshultz ; MD¶ ; ; for the Pediatric Cardiomyopathy Registry Investigators
- 关键词：cardiomyopathy ; echocardiography ; heart failure ; pediatrics
- 刊名：Journal of the American College of Cardiology
- 出版年：15 April, 2014
- 年：2014
- 卷：63
- 期：14
- 页码：1405-1413
- 全文大小：986 K
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聽ventricular (LV) function (fractional shortening or ejection fraction z-score聽<-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; )