Ultrastructural features of the myocardium of children with dilated cardiomyopathy

Abstract

We analyzed the electron-microscopic features of endomyocardial biopsy from pediatric patients with dilated cardiomyopathy (DCM). The specimens examined were taken from the right ventricle of ten patients aged from 2 to 15 years (mean 9.7 years). Biopsy specimens from eight patients with congenital heart disease (tetralogy of Fallot), aged from 3 to 12 (mean 7.3 years), and ten adult patients with DCM, aged from 32 to 60 (mean 45 years), were also examined. Patients considered to have endocardial fibroelastosis, arrhythmogenic right ventricular cardiomyopathy, specific cardiomyopathy, or coronary heart disease were excluded from this study. Specimens from pediatric patients with DCM showed various degrees of ultrastructural abnormalities of myocytes, including myofibrillar fragmentation, mitochondrial abnormalities, and intracellular edema. The ultrastructurally determined contractility failure index based on the severity of myocardial degeneration at the electronmicroscopic level was 4.9 +/- 1.1. This value was significantly higher than that in patients with tetralogy of Fallot (0.9 +/- 0.6, P < 0.001) but was not significantly different from that in adult patients with DCM (6.1 +/- 2.6). The index of pediatric patients with DCM who died within 3 years was high (6.0 +/- 0.8). Basal lamina layering of a capillary (BLL) in the myocardium was revealed in 1 of the 10 (10%) pediatric patients with DCM and in 6 of the 10 (60%) adult patients with DCM (P < 0.05). No BLL was noted in the patients with tetralogy of Fallot. These findings may be related to the pathogenesis of DCM in children and adults.

Focal myocardial fibrosis assessed by late gadolinium enhancement cardiovascular magnetic resonance in children and adolescents with dilated cardiomyopathy

“…Although speculative, the low incidence of myocardial fibrosis assessed by CMR might be related to differences in myocardial remodelling in DCM between children and adults. Nishikawa et al analysed endomyocardial biopsies and found differences in the distribution of microscopic patterns of fibrosis, revealing a higher myocarditic index and a higher incidence of bizarre myocardial hypertrophy as well as discrete ultrastructural differences between adults and children with DCM [ 34 , 36 ] suggesting that factors and mechanisms causing myocardial damage may differ between children and adults. Furthermore, differences in myocardial matrix structure and function [ 37 ] as well as in adrenergic receptor stimulation [ 38 ] have been described, which might have an impact on response to medical therapy thereby influencing ventricular remodelling and prognosis.…”