To investigate the epidemiological characteristics of Kawasaki disease in Jilin province of China and explore its clinical features.

The medical records of children with Kawasaki disease hospitalised in the First Affiliated Hospital of Jilin University and Yanbian University between January, 2000 and December, 2008 were retrospectively analysed.

A total of 735 children with Kawasaki disease were enrolled in this study with 483 boys and 252 girls. The ratio of male to female was 1.92:1. The ages of the children at onset varied from 51 days to 12 years with a mean age of 2.8 years. The children under the age of 5 years accounted for 79.5%, but most children were 2–3 years old. Kawasaki disease occurred all the year and more frequently in both the ending of spring and the beginning of summer. Fever was the most common clinical feature and enlarged cervical lymph nodes were the smallest clinical feature. A cardiovascular lesion was found in 41.4% of these children, in whom coronary artery dilatation was the most common (26.97%). A total of 117 (18.2%) of 643 children (87.5%) receiving intravenous immunoglobulin had a non-response to gamma globulin. Of the 117 children, 66 (56.4%) had cardiovascular lesion. Kawasaki disease recurred in 19 children (2.6%).

The incidence of Kawasaki disease in Jilin province has shown an increasing tendency. The age at onset is slightly higher than that described in other reports. Kawasaki disease is the most common in both the ending of spring and the beginning of summer, and the second incidence peak occurs in autumn.

Although electrical pacing is of great utility in many cardiovascular diseases, its effects on the combined cardiac cell therapy have not been established. We hypothesised that mesenchymal stem cell transplantation changes cardiac sympathetic nerve and gap junction, and concomitant pacing has additional biological effects.

We monitored cardiac rhythm for 4 weeks after human mesenchymal stem cell transplantation (1 × 107, epicardial injection) in 18 dogs in vivo, seven human mesenchymal stem cell with pacing, six human mesenchymal stem cell, and five sham, and evaluated the sympathetic innervation, nerve growth factor-β; tyrosine hydroxylase, angiogenesis, von Willebrand factor, and connexin43 expressions by real time (RT)–polymerase chain reaction and immunostaining. We also measured mRNA expressions of nerve growth factor-β, von Willebrand factor, and connexin43 in vitro culture of human mesenchymal stem cell with or without pacing.

Human mesenchymal stem cell transplanted hearts expressed higher mRNA of nerve growth factor-β (p < 0.01) with sympathetic nerves (p < 0.05), higher mRNA of von Willebrand factor (p < 0.001) with angiogenesis (p < 0.001), but lower mRNA of connexin43 (p < 0.0001) with reduced gap junctions (p < 0.001) than sham. Pacing with human mesenchymal stem cell transplantation resulted in higher expression of mRNA of connexin43 (p < 0.02) and gap junctions (p < 0.001) compared with sham. In contrast, in vitro paced mesenchymal stem cell reduced expression of connexin43 mRNA (p < 0.02).

Human mesenchymal stem cell transplantation increased cardiac sympathetic innervation and angiogenesis, but reduced gap junction after transplanted in the canine heart. In contrast, concomitant electrical pacing increased gap junction expression by paracrine action.

Many studies unequivocally indicate that air pollution is directly linked to the adverse cardiovascular outcomes in the general population. No data are currently available on cardiovascular effects of exposure to trafficked roads in healthy children. Distance of the residence to a major road has been shown to be a useful proxy for long-term traffic exposure and seem to be more consistently associated with atherosclerosis than particulate matter2.5. The aim of this study was to investigate a possible association between the distance to a major road and carotid arterial subclinical markers of atherosclerosis in a group of children in Italy.

The participants consisted of 52 healthy children living in a small town of the Amalphitan Coast with only one highly trafficked road. All children underwent an ultrasound carotid arterial examination.

A statistically significant difference was found in carotid arterial stiffness between children living closer to the main street and other children, both those living between 330 and 730 metres from the main street and those living more than 750 metres from the main street. No significant differences were detectable in carotid arterial thickness and arterial blood pressure among the three groups of children.

This study provides evidence in support of an association of exposure to air pollution with early atherosclerotic markers in healthy children. Impaired vascular health in childhood and adolescence gives further substance to the hypothesis that traffic exhausts are relevant to cardiovascular diseases even early in life.

Improvements in long-term survival of children undergoing the Norwood staged procedure and the arterial switch operation have resulted in the need to prepare these at-risk children for each stage of their developmental trajectory, including school readiness. This study describes and compares functional outcomes following the Norwood staged procedure and arterial switch operations.

This prospective inception cohort study comprised a sample of 73 children (71% boys) who had the Norwood staged procedure (n = 28) or the arterial switch operation (n = 45) at the age of 6 weeks or younger at the Stollery Children’s Hospital, Edmonton, Alberta, between 2002 and 2005. We excluded children who had chromosomal abnormalities or cerebral palsy. When children were 18–24 months of age, parents completed the Adaptive Behavioral Assessment System II. Standard scores for the domains are mean 100, standard deviation (15); skill area scaled scores, 10 (3). Student’s t-test with Bonferonni correction was used to compare groups.

This population has greater than four times the number of children delayed on the General Adaptive Composite than the normative group. Functional outcomes were similar in the two groups other than those of home living (Norwood: 8.8 (2.8) compared with arterial switch: 11.2 (3.1), t = 3.389, p = 0.001) and self-care (Norwood: 5.9 (3.5) versus arterial switch: 8.1 (2.6), t = 3.140, p = 0.002).

These survivors are at increased risk for delayed functional abilities. Self-care, necessary for independence and confidence as children reach school age, was particularly low in the Norwood group. Reasons for low self-care abilities require further study.

This study, which to the best of our knowledge is the first of its kind, aimed to determine the acute and long-term effects of the consumption of grape and pomegranate juices on endothelium function in adolescents with metabolic syndrome, and to compare the effects of these two kinds of juices.

This randomised controlled clinical trial was conducted in 2008 among 30 adolescents, aged 12–15 years, with metabolic syndrome. Participants were randomly assigned to two groups of equal number; one group was asked to drink 18 millilitre per kilogram per day of natural grape juice and the other group was asked to drink 240 millilitre per day of natural pomegranate juice once daily for 1 month. Juices were homemade without any added sweetener. Basal brachial artery dimension and flow-mediated dilation as an index of endothelial function and endothelial-dependent dilation after receiving nitoglycerin spray were evaluated by high-resolution B mode ultrasonography before juice consumption, 4 hours and 30 days after regular daily consumption.

Flow-mediated dilation at 90 seconds and after nitoglycerin significantly improved at 4 hours and at 1 month after drinking both kinds of juices, without significant difference between the two groups. The change at 1 month versus 4 hours was significant only in the grape juice group.

Daily consumption of diets rich in antioxidants might improve endothelial function in adolescents with metabolic syndrome. These effects began as soon as 4 hours after juice consumption. Such beneficial effects should be considered in dietary recommendations for the paediatric age group, notably in obese individuals.

To investigate whether genetic variants in methylenetetrahydrofolate reductase (MTHFR) and methylenetetrahydrofolate dehydrogenase (MTHFD) genes are associated with risk of congenital cardiac disease.

Accumulative evidence suggests that hyperhomocysteinaemia is associated with risk of congenital cardiac disease. Inherited polymorphisms in key folate metabolic pathway genes, MTHFR and MTHFD, may influence the efficiency of folate metabolism and plasma level of homocysteine.

A two-stage case–control study of congenital cardiac disease was conducted by genotyping MTHFR c.1793G>A and four other variants – MTHFR c.677C>T, c.1298A>C, and MTHFD c.1958G>A, c.401C>T – in a Chinese population consisting of 1033 congenital cardiac disease patients and 1067 non-congenital cardiac disease patients.

The variant genotypes of MTHFR c.1793GA/AA were associated with a significantly decreased risk of congenital cardiac disease in two stages combined, with an adjusted odds ratio of 0.67 and a 95% confidence interval of 0.54–0.84 (p = 0.0004). In comparison with wild-type homozygote c.1793GG, the effect was significant in isolated perimembranous ventricular septal defect patients with an adjusted odds ratio of 0.60 and a 95% confidence interval of 0.43–0.83 (p = 0.0003).

These findings indicate that MTHFR c.1793G>A may have a role in susceptibility to sporadic congenital cardiac disease.

Studies show that anti-epileptic drugs increase oxidative stress. Thus, low-density lipoprotein oxidation increases and atherogenesis is induced. Paraoxonase-associated high-density lipoprotein protects low-density lipoprotein and high-density lipoprotein oxidation. The effects of anti-epileptic drugs on paraoxonase activity has not been investigated yet. The aim of this study is to investigate the effect of anti-epileptic drugs on paraoxonase activity, lipid profiles, folat, vitamin B12, homocysteine, thyroid hormones, apolipoprotein A-1, total anti-oxidant capacity, malondialdehyd, nitric oxide, and oxidised low-density lipoprotein. The association with carotid–femoral pulse wave velocity and current biochemical parameters had been searched for assessing the effects of anti-epileptic drugs on the vascular system.

We recruited 59 epileptic patients treated with anti-epileptic drugs and 23 controls (group IV) at least 6 months ago. The epileptic group was divided into three groups by receiving anti-epileptic drugs as follows: group I: carbamazepine, group II: valproic acid, and group III: carbamazepine and valproic acid. Arterial distensibility was assessed with the Complior device.

There was no difference between the current biochemical parameters in epileptic children. Serum-free T4 was decreased, when compared with group IV. Thyroid-stimulating hormone was increased in group II, compared with group IV. The carotid–femoral pulse wave velocity was increased in group III, compared with group IV. The carotid–femoral pulse wave velocity was correlated with thyroid-stimulating hormone and valproic acid levels.

Anti-epileptic drugs may induce atherogenesis by affecting the thyroid hormones. According to the current data, the effects of thyroid hormones on vascular system may be independent of other biochemical markers. Epileptic patients using anti-epileptic drugs must be followed closely for arterial stiffness, and also for the development and progression of atherosclerosis.

The Aristotle basic complexity score and the risk adjustment in congenital cardiac surgery-1 method were developed and used to compare outcomes of congenital cardiac surgery. Both methods were used to compare results of procedures performed on our patients in Croatian cardiosurgical centres and results of procedures were taken abroad.

The study population consisted of all patients with congenital cardiac disease born to Croatian residents between 1 October, 2002 and 1 October, 2007 undergoing a cardiovascular operation during this period.

Of the 556 operations, the Aristotle basic complexity score could be assigned to 553 operations and the risk adjustment in congenital cardiac surgery-1 method to 536 operations. Procedures were performed in two institutions in Croatia and seven institutions abroad. The average complexity for cardiac procedures performed in Croatia was significantly lower. With both systems, along with the increase in complexity, there is also an increase in mortality before discharge and postoperative length of stay. Only after the adjustment for complexity there are marked differences in mortality and occurrence of postoperative complications.

Both, the Aristotle basic complexity score and the risk adjustment in congenital cardiac surgery-1 method were predictive of in-hospital mortality as well as prolonged postoperative length to stay, and can be used as a tool in our country to evaluate a cardiosurgical model and recognise potential problems.

Ectatic aortopathy and arterial abnormalities cause excess morbidity and mortality in Turner syndrome, where a state of vasculopathy seemingly extends into the major head and neck branch arteries.

We investigated the prevalence of abnormalities of the major intrathoracic arteries, their interaction with arterial dimensions, and their association with karyotype.

Magnetic resonance imaging scans determined the arterial abnormalities as well as head and neck branch artery and aortic dimensions in 99 adult women with Turner syndrome compared with 33 healthy female controls. Echocardiography determined aortic valve morphology.

In Turner syndrome, the relative risk of any congenital abnormality was 7.7 (p = 0.003) and 6.7 of ascending aortic dilation (p = 0.02). A bovine aortic arch was seen in both Turner syndrome and controls. Other abnormalities were only encountered in Turner syndrome: elongated transverse aortic arch (47%), bicuspid aortic valve (27%), aortic coarctation (13%), aberrant right subclavian artery (8%), and aortic arch hypoplasia (2%). The innominate and left common carotid arteries were enlarged in Turner syndrome (p < 0.001). Significant associations were first, bicuspid aortic valve with aortic coarctation, elongated transverse aortic arch, and ascending aortic dilation; second, aortic coarctation with elongated aortic arch and descending aortic dilation; third, 45,X with aortic coarctation, elongated transverse aortic arch and ascending aortic dilation; and fourth, branch artery dilation with bicuspid aortic valve, aortic coarctation, elongated transverse aortic arch and 45,X.

An increased risk of arterial abnormalities, aortic dilation, and enlargement of the branch arteries was found in Turner syndrome without distinct patterns of co-segregation.

The aim of the study is, by comparing cardiac parameters between children native to 1890 metres with children living at sea level, to find out whether there is any impairment in cardiac function related to that altitude.

Electrocardiographic, conventional, and tissue Doppler echocardiographic parameters were compared in 42 healthy children native to 1890 metres, and in 21 healthy age and gender matched children living at sea level. Plasma haemoglobin level and oxygen saturation measured by pulse oxymeter were also obtained from all patients.

Haemoglobin levels were higher, and oxygen saturation levels were lower in children native to 1890 metres. Conventional echocardiographic parameters and mitral annular myocardial parameters were all similar between children native to 1890 metres and children living at sea level. Tricuspid lateral annular early diastolic velocity and the ratio of early-to-late diastolic velocity were significantly lower and tricuspid lateral annular izovolumetric relaxation time was significantly higher in children native to 1890 metres than children living at sea level.

Children living at 1890 metres of altitude predispose to asymptomatic right ventricular diastolic dysfunction or otherwise they remain as healthy children.

To describe the cardiac lesions seen in children with trisomy 21, the outcome of these children and rates of access to corrective surgery at the Bustamante Hospital for Children.

A 10-year retrospective review of the records of trisomy 21 patients with cardiac lesions referred to the Bustamante Hospital for Children was conducted.

A total of 76 patients were enrolled in the study, 30 (40%) males and 46 (60%) females; among these 110 cardiac lesions were detected. A total of 20 (26%) patients died, 48 (63%) survived, and for 8 (11%) the status was unknown. The most common lesion was the atrioventricular septal defect, which accounted for 41 (37%) of all the lesions and occurred as a single defect in 24 (53%) patients. At presentation, 33 (46%) patients had one or more medical complication; 30 (91%) had cardiac failure, 10 (30%) had pneumonia and 5 (15%) had evidence of systemic pulmonary arterial pressures. Cardiac catheterisation was recommended for 43 (56%) patients but only 10 (23%) had the procedure done. Surgery was recommended for 60 (79%) patients; of these 6 (10%) patients had the procedure done. The median time of survival was 88 months (7.3 years). The age of presentation was not found to significantly affect outcome.

Trisomy 21 patients with cardiac lesions have high morbidity and mortality. This morbidity and mortality could be reduced if surgical intervention was offered routinely.

To determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification.

Retrospective study of 41 consecutive children affected by dilated cardiomyopathy – aged 0–14 years; median 33.4 plus or minus 49.25 – between 1993 and 2008. We reviewed the medical history to determine age at diagnosis, family history, previous viral illness, aetiology, symptoms and signs at presentation, treatment, and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. We also carried out a metabolic evaluation including blood lactate, pyruvate, carnitine, amino acids, urine organic acids, assessment of respiratory chain enzymes, and analysis of histopathological material. Survival curves were constructed by the Kaplan–Meier method.

Follow-up ranged from 10 days to 162 months – median 45.25 plus or minus 41.15 months. Freedom from death or cardiac transplantation was 68.3% at 5 years. The primary end-point of death/cardiac transplantation was associated with the need for intravenous inotropic support. A trend towards a poorer prognosis was found for age at diagnosis of more than 5 years and for a metabolic aetiology of dilated cardiomyopathy. For the children affected by cardiomyopathy as part of a multi-system involvement, mortality was 50%.

In children, dilated cardiomyopathy is a diverse disorder with outcomes that depend on cause, age, and cardiac failure status at presentation. Overt cardiac failure at presentation is a major prognostic factor for death or cardiac transplantation. Older age at presentation and metabolic aetiology may be associated with a poorer prognosis.

The goal of this study was to see whether the open anastomosis technique using vacuum-assisted venous drainage at the time of the Fontan procedure was associated with decreased post-operative pleural effusion.

We analysed a subgroup of patients with a functional single ventricle who underwent non-fenestrated total cavopulmonary connection completion with the insertion of an extracardiac conduit as the sole or predominant procedure conducted by a single surgeon at a single institute, using either an open or closed anastomosis technique.

Median age and weight were 2.3 years, with a range from 1.3 to 27.6 years and 11.4 kilograms, with a range from 9.7 to 43 kilograms, respectively. The open anastomosis technique was associated with a shorter bypass run (p = 0.015), decreased surgical duration (p = 0.032), fewer pleural effusion days (p = 0.049), and lesser pleural effusion (p = 0.013) than closed anastomosis. Correlation analysis demonstrated a significant relationship between the amount of pleural effusion and surgical duration (correlation efficient, 0.535; p = 0.033). A logistic regression model showed that the open technique was associated with a 20-fold increase in the likelihood of having a total chest tube discharge of less than 300 millilitres (p = 0.027).

The open anastomosis technique shortens operative duration and bypass run, which in turn might contribute to decreased pleural effusion soon after the modified Fontan procedure.