The prognosis in children with idiopathic dilated cardiomyopathy (IDC) is guarded, with the 5-year mortality rate ranging from 14% to 50%, owing to sudden cardiac death (SCD) or pump failure. The risk factors for SCD in adults with IDC include asymptomatic nonsustained ventricular tachycardia and poor left ventricular function. It is unclear whether these findings can be extrapolated to the pediatric population. A retrospective review of all patients with the diagnosis of IDC seen at a single institution from 1990 to 2004 was performed. A total of 85 patients (46 males), with a median age of 3.8 years (0 days to 17.3 years) were studied. The mean left ventricular ejection fraction was 25 +/- 12% (median 23%, range 45% to 45%) at presentation. The following arrhythmias occurred at presentation or during the initial hospitalization: nonsustained ventricular tachycardia in 6, sustained ventricular tachycardia or fibrillation in 1, supraventricular arrhythmias in 6, and both atrial and ventricular arrhythmias in 1. During a subsequent hospitalization or outpatient follow-up, 7 patients had the following arrhythmias: supraventricular arrhythmias in 2, nonsustained ventricular tachycardia in 4, and both atrial and ventricular arrhythmias in 1. The cumulative survival rate was 40% at a mean follow-up of 6.2 years (95% confidence interval 4.4 to 8.1). One single episode of SCD occurred in 1 patient without a history of sustained arrhythmias. In conclusion, in children with IDC, despite the low left ventricular ejection fraction and presence of ventricular arrhythmias, only one episode of SCD occurred in this group of patients. Given the 1% incidence of SCD in this cohort, the use of implantable cardioverter-defibrillators as primary prevention in children with IDC might not be indicated.
"Inflammatory dilated cardiomyopathy (DCMi) is among the most common causes of noncon genital heart failure in individuals under the age of 40 (Dimas et al., 2009). There has been only limited success with symptomatic therapy in chronic DCMi patients, leaving cardiac tran splantation the only cure for end stage heart failure secondary to DCMi (Pietra et al., 2012). "
[Show abstract][Hide abstract] ABSTRACT: Inflammatory dilated cardiomyopathy (DCMi) is a major cause of heart failure in individuals below the age of 40. We recently reported that IL-17A is required for the development of DCMi. We show a novel pathway connecting IL-17A, cardiac fibroblasts (CFs), GM-CSF, and heart-infiltrating myeloid cells with the pathogenesis of DCMi. Il17ra(-/-) mice were protected from DCMi, and this was associated with significantly diminished neutrophil and Ly6Chi monocyte/macrophage (MO/MΦ) cardiac infiltrates. Depletion of Ly6Chi MO/MΦ also protected mice from DCMi. Mechanistically, IL-17A stimulated CFs to produce key chemokines and cytokines that are critical downstream effectors in the recruitment and differentiation of myeloid cells. Moreover, IL-17A directs Ly6Chi MO/MΦ in trans toward a more proinflammatory phenotype via CF-derived GM-CSF. Collectively, this IL-17A-fibroblast-GM-CSF-MO/MΦ axis could provide a novel target for the treatment of DCMi and related inflammatory cardiac diseases.
Journal of Experimental Medicine 06/2014; 211(7):1449-64. DOI:10.1084/jem.20132126 · 12.52 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The purpose of this study was to establish the incidence of and risk factors for sudden cardiac death (SCD) in pediatric dilated cardiomyopathy (DCM).
The incidence of SCD in children with DCM is unknown. The ability to predict patients at high risk of SCD will help to define who may benefit most from implantable cardioverter-defibrillators.
The cohort was 1,803 children in the PCMR (Pediatric Cardiomyopathy Registry) with a diagnosis of DCM from 1990 to 2009. Cumulative incidence competing-risks event rates were estimated. We achieved risk stratification using Classification and Regression Tree methodology.
The 5-year incidence rates were 29% for heart transplantation, 12.1% non-SCD, 4.0% death from unknown cause, and 2.4% for SCD. Of 280 deaths, 35 were SCD, and the cause was unknown for 56. The 5-year incidence rate for SCD incorporating a subset of the unknown deaths is 3%. Patients receiving antiarrhythmic medication were at higher risk of SCD (hazard ratio: 3.0, 95% confidence interval: 1.1 to 8.3, p = 0.025). A risk stratification model based on most recent echocardiographic values had 86% sensitivity and 57% specificity. Thirty of 35 SCDs occurred in patients who met all these criteria: left ventricular (LV) end-systolic dimension z-score >2.6, age at diagnosis younger than 14.3 years, and the LV posterior wall thickness to end-diastolic dimension ratio <0.14. Sex, ethnicity, cause of DCM, and family history were not associated with SCD.
The 5-year incidence rate of SCD in children with DCM is 3%. A risk stratification rule (86% sensitivity) included age at diagnosis younger than 14.3 years, LV dilation, and LV posterior wall thinning. Patients who consistently meet these criteria should be considered for implantable cardioverter-defibrillator placement.
Journal of the American College of Cardiology 02/2012; 59(6):607-15. DOI:10.1016/j.jacc.2011.10.878 · 16.50 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Dilated cardiomyopathy is a serious and life-threatening disorder in children. It is the most common form of pediatric cardiomyopathy. Therapy for this condition has varied little over the last several decades and mortality continues to be high. Currently, children with dilated cardiomyopathy are treated with pharmacological agents and mechanical support, but most require heart transplantation and survival rates are not optimal. The lack of common treatment guidelines and inadequate survival rates after transplantation necessitates more therapeutic clinical trials. Stem cell and cell-based therapies offer an innovative approach to restore cardiac structure and function towards normal, possibly reducing the need for aggressive therapies and cardiac transplantation. Mesenchymal stem cells and cardiac stem cells may be the most promising cell types for treating children with dilated cardiomyopathy. The medical community must begin a systematic investigation of the benefits of current and novel treatments such as stem cell therapies for treating pediatric dilated cardiomyopathy.
Current Cardiology Reports 06/2013; 15(6):369. DOI:10.1007/s11886-013-0369-z · 1.93 Impact Factor
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