Cost-Utility of Wearable Cardioverter Defibrillators in Children with Dilated Cardiomyopathy during Medical Optimization: Is it Worth the Wait?
Dilated cardiomyopathy (DCM) is the most common form of cardiomyopathy in both adults and children, and the estimated cost of caring for patients with this disease is in the order of billions of dollars annually in the United States alone. Although in adults, the etiology is usually related to coronary artery disease, in children, the mechanisms are far more varied, and the course is less well described and likely dependent on cause and presentation.1 An important facet of the management paradigm for these children includes approach to sudden death prevention, an area that continues to lack substantial data.
Contributors : Li Wang ; Peng Yu ; Zheng Li ; Zongna RenSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensWe studied the cell compositon of two types of human heart disease (coronary atherosclerotic heart disease and dilated cardiomyopathy) by single-cell sequencing. Distinct subgroups of cardiac muscle, fibroblast cell and endothelial cell were detected. We generated a cell-cell interaction network using specific expressed ligands and receptors of cells. And we also observed the change of interaction and cell transformation with age.
Abstract Dilated cardiomyopathy (DCM) is the most common cause of heart failure (HF) in children, resulting in high mortality and need for heart transplantation. The pathophysiology underlying pediatric DCM is largely unclear, however there is emerging evidence that molecular adaptations and response to conventional HF medications differ between children and adults. In order to gain insight into alterations leading to systolic dysfunction in pediatric DCM, we measured cardiomyocyte contractile properties and sarcomeric protein phosphorylation in explanted pediatric DCM myocardium (N=8) compared to non-failing (NF)...
In this edition of the Journal, Li et al report on patients with dilated cardiomyopathy (DCM) and arrhythmias (DCM-A) compared with patients with DCM with no arrhythmias (DCM-NA), with the explicit intent to identify the genetic causes of arrhythmias in DCM.1 Using whole-exome sequencing and high-depth targeted next-generation sequencing followed by Sanger sequencing of all causative variants to eliminate false-positive results, eight specific DCM-A pedigrees and two separate cohorts of 1232 consecutive unrelated sporadic DCM-NA patients were studied (550 patients in discovery cohort, 682 patients in replication cohort).
Publication date: Available online 17 October 2019Source: Nutrition, Metabolism and Cardiovascular DiseasesAuthor(s): Xiao-lan Chen, Xue-biao Wei, Jie-leng Huang, Zu-hui Ke, Ning Tan, Ji-yan Chen, Yuan-hui Liu, Dan-qing YuAbstractBackground and AimsThe prognostic nutritional index (PNI) had been associated with adverse outcomes in numerous clinical conditions. However, its influence on idiopathic dilated cardiomyopathy (DCM) was not determined. This aim of this study was to determine the predictive ability of PNI in patients with idiopathic DCM.Methods and ResultsA total of 1,021 consecutive patients with idiopathic DCM we...
To recapitulate progressive human dilated cardiomyopathy (DCM) and heart block in the Lmna R225X mutant mice model and investigate the molecular basis of LMNA mutation induced cardiac conduction disorders (CD); To investigate the potential interventional impact of exercise endurance.
We present a retrospective review of 13 consecutive children who underwent implantation of VAD between 2001 and 2018 in our center. The median age was 12 years (1–17 years), weight was 45 kg (10–82 kg). Etiologies of heart failure were dilated cardiomyopathy (CMP) (n = 8), myocarditis (n = 2), ischemic CMP (n = 1), restrictive CMP (n = 1) and congenital heart disease (n = 1). Pre-implantation ECMO was used in 5, mechanical ventilation in 4, renal replacement therapy in 2 and IABP in 1. Devices used were: Berlin Heart EXCO...
The prognostic nutritional index (PNI) had been associated with adverse outcomes in numerous clinical conditions. However, its influence on idiopathic dilated cardiomyopathy (DCM) was not determined. This aim of this study was to determine the predictive ability of PNI in patients with idiopathic DCM.
The indications for mitral valve surgery in patients with advanced non-ischemic dilated cardiomyopathy and functional mitral regurgitation are still unclear. We reviewed our experience of mitral valve surgery in such patients and explored factors that were predictive of outcomes. Fifty-three consecutive patients with non-ischemic dilated cardiomyopathy and an ejection fraction
We present two young patients (
ConclusionsWe describe a unique and novel cellular model that provides insight into the mitochondrial abnormalities present in DCMA and identifies SS-31 as a potential therapeutic for this devastating disease.