Recovery of Echocardiographic Function in Children With Idiopathic Dilated Cardiomyopathy

Abstract

Objectives To determine the incidence of and predictors for 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 less than 18 years old in the Pediatric Cardiomyopathy Registry who had both depressed left ventricular (LV) function (fractional shortening [FS] or ejection fraction [EF] z-score <−2) and LV dilation (end-diastolic dimension [LVEDD] z-score >2) at diagnosis and who had at least one 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 inclusion criteria, 741 (85%) had both echocardiograms. At 2 years, 22% had recovered normal LV function and size; 51% had died or undergone heart transplant (median, 3.2 months), and 27% had persistently abnormal echocardiograms. Younger age (hazard ratio, 0.90; 95% CI, 0.86 to 0.95) and lower LVEDD z-score (0.75; 95% CI, 0.68 to 0.84) independently predicted normalization. Nine children (9%) with normal LV function and size within 2 years of diagnosis later underwent heart transplant 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 for recovery, such as gene 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. Clinical Trials Registration # NCT00005391

Risk Factors for Death or Heart Transplant for Patients with Right Ventricular Dysfunction After the Norwood Procedure: A Secondary Analysis of the Single Ventricle Reconstruction Trial

“…In other groups of patients such as the dilated cardiomyopathy cohort, myocardial recovery is a well-documented phenomenon (Everitt et al 2014;Alexander et al 2013) patients were able to proceed to stage II. This is demonstrating that myocardial recovery is possible in this population as well.…”

“…A more dilated ventricle that works harder for the same result would result in a decreased circulatory reserve. The physiology associated with ventricular dilation might explain why this is an independent risk factor for death or transplant in multiple other cardiac conditions (Alexander et al 2012, Abe et al 2013, Everitt et al 2014, Therrien et al 2005). Figure 37.…”

“…Therefore, ventricular dilatation is usually observed when there is significant ventricular dysfunction. (Matsumura et al 2013;Alexander et al 2013) In other diseases, such as dilated cardiomyopathy, ventricular dilatation is one of the main predictors of outcome where ejection fraction is often not an important predictor (Everitt et al 2014;Alexander et al 2013). In other congenital diseases, such as Tetralogy of Fallot, right ventricular dilatation has also been shown to be one of the main outcome drivers (Therrien et al 2005;Henkens et al 2007).…”

Pediatric cardiomyopathies: A review of literature on clinical status and meta-analysis of diagnosis and clinical management methods

“…Sixteen (16) prospective or retrospective studies meeting the inclusion criteria were included in this meta-analysis [12,24,26,30,31,[54][55][56][57][58][59][60][61][62][63][64]. Altogether, the studies investigated a total sample of 6,665 patients aged younger than 18 years with pediatric cardiomyopathies having mean high mortality rate of 12% within five years of diagnosis.…”

“…Altogether, the studies investigated a total sample of 6,665 patients aged younger than 18 years with pediatric cardiomyopathies having mean high mortality rate of 12% within five years of diagnosis. The study period ranged from 1958 [12] to 2012 [61]. Echocardiographic defined left ventricular dysfunction (also used in the recruitment of pediatric patients constituting the PCMR data), was the dominant method of diagnosis adopted by 14 studies (87.5%) [12,24,26,30,31,[54][55][56][59][60][61][62][63][64].…”