[Changes in serum chromogranin A and urotensin II levels in children with chronic heart failure].

OBJECTIVE: To examine the changes in serum chromogranin A (CgA) and urotensin II (U II) levels in children with chronic heart failure (CHF) and their clinical significance. METHODS: A total of 58 children with CHF, among whom 17 had endocardial fibroelastosis (EFE) and 41 had dilated cardiomyopathy (DCM), were selected as CHF group, and 20 healthy children were selected as control group. Serum levels of CgA and U II were measured using enzyme-linked immunosorbent assay, and the level of N-terminal pro-brain natriuretic peptide (NT-proBNP) was determined by bi-directional lateral flow immunoassay. Ventricular remodeling indices were measured using echocardiography. The correlation between serum CgA and U II levels and ventricular remodeling was evaluated by Pearson correlation or Spearman's rank correlation analysis. RESULTS: There were no significant differences in serum CgA and NT-proBNP levels between children with grade II heart function and the control group (P>0.05). However, the serum CgA and NT-proBNP levels gradually increased as the heart function grade increased, and were significantly higher in grade III and IV children compared to those in the control group (P<0.05). U II levels were lower in children with grade II, III, or IV heart function than those in the control group (P<0.05), and significantly decreased with the aggravation of CHF (P<0.05). There were no significant differences in CgA and U II levels between patients with EFE and DCM (P>0.05). Serum CgA concentration was positively correlated with left ventricular mass index (LVMI), NT-proBNP, and cardiac function classification (r=0.279, 0.649, and 0.778 respectively; P<0.05), but was negatively correlated with left ventricular ejection fraction (LVEF), left ventricular fractional shortening (LVFS), and U II (r=-0.369, -0.322, and -0.718 respectively; P<0.05). Serum U II concentration was negatively correlated with NT-proBNP and cardiac function classification (r=-0.472 and -0.591 respectively; P<0.05), but was not correlated with LVMI, LVEF, and LVFS (P>0.05). CONCLUSIONS: CgA may play a role in ventricular remodeling in children with CHF. Serum CgA and U II may serve as a reference for the diagnosis and functional classification of heart failure.

[Changes of heart-type fatty acid-binding protein in children with chronic heart failure and its significance].

OBJECTIVE: To study serum levels of heart-type fatty acid-binding protein (h-FABP) in children with chronic heart failure (CHF), and the correlation between heart function and the level of h-FABP, with the aim of studying the significance of h-FABP in CHF. METHODS: Thirty-six children with CHF, including 16 cases of endocardial fibroelastosis (EFE) and 20 cases of dilated cardiomyopathy (DCM) were enrolled in the study. Thirty healthy children sevred as the control group. Serum levels of h-FABP were determined using ELISA, and left ventricular ejection fraction (LVEF), cardiac index (CI) and fractional shortening of the left ventricle (LVSF) were measured by two-dimensional echocardiography in the CHF group. RESULTS: Mean levels of h-FABP in the CHF group were significantly higher than in the control group (21.7±4.3 ng/mL vs 6.2±1.7 ng/mL; P<0.01). The worse the heart function, the higher the h-FABP levels (P<0.01). Mean levels of h-FABP in both the EFE and DCM groups were significantly higher than in the control group (P<0.01). Serum h-FABP concentrations were negatively correlated with LVEF, CI and LVSF (r=-0.65, -0.64 and -0.71 respectively; P<0.01) in the CHF group. CONCLUSIONS: Serum h-FABP levels increase in children with CHF and are closely related to the severity of the condition. Serum h-FABP levels can be used as a biomarker for the diagnosis of heart failure and the evaluation of its severity.

Myocardial tumor necrosis factor-alpha expression in a sudden death due to dilated cardiomyopathy with endomyoelastofibrosis.

During the clinical course of dilated cardiomyopathy, arrhythmias, thromboembolism and sudden death are common and may occur at any stage. In patients with dilated cardiomyopathy, elevated plasma levels of tumor necrosis factor-alpha are associated with poor prognosis. In animal experiments and clinical trials, myocardial tumor necrosis factor-alpha expression correlates with increased mortality rates. A case of sudden death of a previously asymptomatic young man, having a definitive autoptic diagnosis of dilated cardiomyopathy with endomyoelastofibrosis, is presented. We investigated the cardiac morphology and the expression of tumor necrosis factor-alpha in cardiac tissue specimens to elucidate the role of tumor necrosis factor-alpha expression in this fatal case. Our results demonstrate a strong positive myocytes reaction for tumor necrosis factor-alpha in the heart specimens. The pathogenesis of the fatal event is discussed with particular reference to the histological findings and myocytes expression of tumor necrosis factor-alpha.

[Endocardial fibrosis in neuroendocrine tumors of the gastroenteropancreatic system]. / Endokardfibrose bei neuroendokrinen Tumoren des gastroenteropankreatischen Systems.

Clinical characteristics, echocardiographic and Doppler echocardiographic findings, as well as serum levels of chromogranin A were recorded on 62 patients (27 women, 35 men; mean age 55 [11-83] years) with histologically confirmed tumours of the gastroenteropancreatic (GEP) system. Changes in the right heart were found in 14 patients (22%), club-like thickening of tricuspid leaflets in 13, tricuspid regurgitation in 14, stenosis in 2 and right atrial or right ventricular dilatation in 11 and 5, respectively. There was no difference between the patients with or without right-heart changes in regard to age, presence of carcinoid syndrome, duration of symptoms, primary tumour site, pattern of metastases, treatment or chromogranin A level. Two patients with nonfunctioning tumours had right-heart changes. While clinical and biochemical parameters did not identify patients with right-heart changes, echocardiography demonstrated all haemodynamically significant endocardial changes. Even patients with nonfunctioning GEP tumours should be regularly monitored by echocardiography.

Inheritance of familial primary endocardial fibroelastosis.

Two related families with 15 children, seven of whom developed endocardial fibroelastosis (EFE) are described. Three of the children died during infancy, and the disease was confirmed in one of them at autopsy. The survivors, two sisters age 5 years and 15 months (Family A) and two sisters age 17 and 14 years (Family B), are now symptomless and show a decrease in left ventricular hypertrophy. The mode of inheritance of EFE in our two families appears to be either autosomal or X-linked dominant with reduced penetrance.