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In children with mitochondrial disease, cardiomyopathy was common (17%) and was associated with increased mortality. The prognosis for children with cytochrome-c oxidase deficiency and cardiomyopathy appeared to be particularly unfavorable.

Background Despite advances in pediatric health care over recent decades, it is not clear whether survival in children with congenital heart disease (CHD) is still increasing. Methods and Results We identified all patients with CHD using nationwide Swedish health registries for 1980 to 2017. We examined the survival trends in children with CHD; we investigated the mortality risk in patients with CHD compared with matched controls without CHD from the general population using Cox proportional regression models and Kaplan–Meier survival analysis. Among 64 396 patients with CHD and 639 012 matched controls without CHD, 3845 (6.0%) and 2235 (0.3%) died, respectively. The mean study follow‐up (SD) was 11.4 (6.3) years in patients with CHD. The mortality risk was 17.7 (95% CI, 16.8–18.6) times higher in children with CHD compared with controls. The highest mortality risk was found during the first 4 years of life in patients with CHD (hazard ratio [HR], 19.6; 95% CI, 18.5–20.7). When stratified by lesion group, patients with non‐conotruncal defects had the highest risk (HR, 97.2; 95% CI, 80.4–117.4). Survival increased substantially according to birth decades, but with no improvement after the turn of the century where survivorship reached 97% in children with CHD born in 2010 to 2017. Conclusions Survival in children with CHD has increased substantially since the 1980s; however, no significant improvement has been observed this century. Currently, >97% of children with CHD can be expected to reach adulthood highlighting the need of life‐time management.

The results suggest that routine use of intraoperative thromboelastometry in pediatric cardiac surgery to guide transfusions is associated with a reduced proportion of patients receiving transfusions and an altered transfusion pattern.

Aim: To study whether natriuretic peptide types B (BNP) and A (ANP) reflect clinical signs of heart failure (CSHF) in children with congenital heart defects or cardiomyopathy resulting in different types of haemodynamic situations, such as pressure overload in coarctation of the aorta (CoA), volume overload in ventricular septal defect (VSD) or systolic dysfunction in dilated cardiomyopathy (DCM). Methods: Blood samples for plasma P‐BNP and P‐ANP were taken before procedures during regular investigation from 26 children (9 CoA, 11 VSD and 6 DCM). The ordinary paediatric cardiologist performed the cardiac evaluation and the data were retrieved from medical charts. CSHF was considered positive if two of the following criteria were fulfilled: reduced physical capacity, feeding disorders, dyspnoea, tachypnoea, hepatomegaly and oedema. The statistical methods were non‐parametric. Results: 0/9 children with CoA, 5/11 with VSD and 6/6 with DCM had CSHF. In children with CSHF, P‐BNP and P‐ANP were higher, 263 ng l−1 (range 47.5–1300) and 303 ng l−1 (range 168–466), than in those without CSHF, 12.3 ng l−1 (range 4.8–30.8) and 42.9 ng l1 (range 13.7–189), respectively (p < 0.001, Mann‐Whitney U‐test), irrespective of the diagnosis. The same relationship was also found in the group of children with VSD. Conclusion: Plasma levels of ANP and BNP increase in children with CSHF. This increase is seen irrespective of whether it is due to systolic dysfunction, as in children with DCM, or to a volume overload with a normal systolic function, as in children with VSD.

Increasing interest has been directed toward the possible role of trophically acting molecules as modulators or initiators, or both, of myocardial hypertrophy. The aim of the present study was to investigate the possible role of one such molecule, namely, insulin-like growth factor I, in myocardial hypertrophy developed in response to renal artery stenosis. Two-kidney, one clip Goldblatt hypertension was induced in Wistar rats weighing 180 g, and sham-operated animals were used as controls. Blood pressure was increased as early as 2 days after clipping (133 ±4 versus 116±4 mm Hg, p<0.05), and the increase persisted 4 and 7 days after clipping (148±6 versus 129±3 mm Hg, p<0.01 and 171±5 versus 139±3 mm Hg,p<0.01, respectively). Left ventricular weight followed a similar pattern (373±7 versus 350±8 mg, NS, 415±11 versus 386±9 ing,/><0.01, and 466±11 versus 391 ±10 mg,/><0.01 at 2, 4, and 7 days after clipping, respectively), but no changes in body weight between the groups were observed. Insulin-like growth factor I messenger RNA (mRNA) was quantified using a solution hybridization assay. W hen challenged by a chronically increased systemic pressure load, the heart responds with an adaptive left ventricular hypertrophy to normalize wall stress and myocardial oxygen consumption (see Friberg 1 ). However, the precise mechanism for transforming the mechanical stimuli of increased pressure or volume work into the hypertrophic response is not known. In addition to mechanical stimuli, different trophic factors such as catecholamines and angjotensin II may influence the From the Departments of Physiology (H.W., J.I., P.F.) and Histology

Obese adolescents had a higher LVM than age-matched lean subjects, which correlated mainly with body mass index and systolic blood pressure. These findings add to the established cardiovascular risk profile of obese adolescents.

We analyzed the natriuretic peptide type B (BNP) and A (ANP) levels in the plasma of 38 children with stable functionally univentricular heart defects, 10 of whom had undergone the first palliative step (Shunt), 13 of whom had undergone the second palliative step (Glenn), and 15 of whom had completed total cavopulmonary connection (TCPC). The levels of BNP and ANP were significantly higher after the first palliative step, 31.6 ng/l (8.3-122) (median and range) and 101.2 ng/l (17.1-203), respectively, than after the Glenn procedure, 6.7 ng/l (0.0-16.0) and 23.6 ng/l (15.7-54.4), respectively, as well as after completed TCPC, 9.0 ng/l (0.0-39.1) and 20.9 ng/l (11.2-28.3), respectively, and, in a control group of children without heart defects, 5.8 ng/l (0.0-38.7) and 32.9 ng/l (11.7-212.1), respectively (p = 0.0003 and p = 0.0003, respectively). After the first palliative step, the BNP and ANP levels were the same in children with right and left ventricular morphology (p = 0.67 and p = 0.52, respectively). After unloading the ventricle (Glenn and TCPC together), BNP levels were higher in children with right ventricular morphology compared with those with left ventricular morphology and controls (p = 0.02). Children with functionally univentricular hearts in stable condition have increased BNP and ANP levels after the first palliative procedure. After the second and third palliative steps, the BNP and ANP levels were low and similar to those of children without heart defects. However, BNP levels in children with a systemic ventricle of right ventricular morphology were higher than those in children with left ventricular morphology.