There is limited data available on characteristics of profiles of lipids in children. The purpose of our investigation, therefore, was to determine the lipid profile, and analyze the prevalence of dyslipidaemia, in subgroups of children according to different gender, districts of residence, and ages in Beijing, China.

We included 18,944 school children, aged from 7 to 18 years, in our community-based cross-sectional study, measuring the levels of total cholesterol and triglyceride in capillary blood, and at the same time determining their weight and height. Weights, heights, circumference at the waist, and body mass index proved to be significantly greater for the boys than the girls. The levels of total cholesterol of boys and girls were 3.98 ± 0.35, and 4.02 ± 0.35 mmol/L, respectively, while the comparable levels of triglycerides were 1.08 ± 0.52, and 1.18 ± 0.66 mmol/L, respectively. The percentages of hypercholesterolaemia, hypertriglyceridaemia, combined dyslipidaemia, and hyperlipidaemia were 1.2, 8.8, 0.4, and 9.7%, respectively. The incidences of hypertriglyceridaemia, combined dyslipidaemia, and hyperlipidaemia of girls were higher than boys. The levels of triglycerides for boys aged from 11 to 18 years living in an urban setting were higher than those for boys living in rural settings, as were the levels of total cholesterol for boys aged from 12 to 16 years.

Our study provides the newest current profiles of lipids for children living in Beijing. We found significant influences of age, districts of residence, and genders on the levels of lipids, features which need further attention in the prevention and treatment of dyslipidaemia.

Patients with Turner’s syndrome are at risk of aortic dilation and dissection. Currently, it is not known whether such dilation is related to associated cardiovascular abnormalities, or to the genetic anomaly itself.

We studied echocardiographically 107 patients with genetically proven Turner’s syndrome, with heterogeneous underlying karyotypes, and without associated cardiac lesions. Their average age was 19.6 plus or minus 8.4 years. We compared the finding with those from 71 age-matched healthy female volunteers. The diameter of the aorta was measured at the level of the basal attachments of the aortic valvar leaflets, the sinuses of Valsalva, the sinutubular junction, and its ascending component.

Compared to control subjects, the patients with Turner’s syndrome had larger diameters of the aorta at the level of the sinuses of Valsalva, at 23.4+/−4.8 versus 25.5+/−4.1 millimetres (p = 0.0014), the sinutubular junction, at 19.9+/−3.8 versus 23.3+/−4.1 millimetres (p < 0.0001), and the ascending aorta, at 22.3+/−4.9 versus 24.6+/−4.4millimetres (p = 0.0011). Dilation of the sinutubular junction, found in just over one-quarter of the patients, was more common than dilation of the ascending aorta, the latter found in less than one-tenth. The patients with Turner’s syndrome, therefore, presented with remodelling of the aortic root, with relative dilation of the sinutubular junction. The underlying karyotype influenced both the dimensions of the sinutubular junction (p = 0.0054), and the ascending aorta (p = 0.0064), so that patients with the karyotype 45X had larger aortas. The karyotype was the strongest predictor by multivariate analysis for dilation at both these sites (p = 0.0138 and 0.0085, respectively).

Dilation at the sinutubular junction is frequent in patients with Turner’s syndrome, and is more common than dilation of the ascending aorta. The syndrome is associated with a remodelling of the aortic root, with prominent dilation of the sinutubular junction. There seems to be a relation between aortic dilation and the underlying genotype.

Exercise echocardiography assesses exercise-induced pulmonary hypertension. The upper normal limit of right ventricular systolic pressure during exercise is not well established. Our study aims to investigate the response of right ventricular systolic pressure in relation to aerobic capacity.

Cardiopulmonary exercise testing using a treadmill, and echocardiography during supine cycling, were performed in 113 healthy volunteers aged 13 to 25 years. Maximal right ventricular systolic pressure during evaluable exercise studies obtained in 108 subjects showed a Gaussian distribution only after separating the endurance trained subjects, specifically 12 athletes with Z-score of peak oxygen uptake higher than 2.0, from the normally trained group of 97 subjects. Maximal right ventricular systolic pressure during exercise in the normally trained group showed a mean of 38.0 millimetres of mercury, with standard deviation of 7.2, a median value of 39.0, and a range from 17 to 63, and the 95th percentile was 51 millimetres of mercury. In the athletes, the maximal right ventricular systolic pressure was higher, with a median of 55.5, a range from 28 to 69, this being significant, with p equal to 0.004). Of the 12 athletes, 8 (67%) showed a response of right ventricular systolic pressure to exercise exceeding 50 millimetres of mercury, but only 8 of 97 normally trained subjects (8%) showed a similar response, this also being significant, with p less than 0.001.

Our study confirms the great variability in the response of right ventricular systolic pressure to exercise in healthy individuals, with 50 millimetres of mercury representing the upper normal limit. Endurance-trained athletes show higher levels, and two-thirds have abnormal responses exceeding 50 millimetres of mercury.

To analyze cardiac involvement and its progression in mucopolysaccharidoses, and to assess the short term impact of new therapeutic strategies.

We studied echocardiographically 57 patients with various types of mucopolysaccharidoses, specifically types I, II, III, IV and VI, with a median age at the diagnosis of cardiac involvement of 5 years, following them for a median of 4.6 years, with a range from 0.9 to 21.2 years. We used a scoring system, along with the so-called delta score, to quantify the severity of involvement at baseline and at last examination, and to chart their progression over time.

Cases with cardiac involvement increased from 59.6% to 87.3% at the last examination. The scores increased with age, and were significantly different according to the specific type of mucopolysaccharidosis. Involvement of the mitral valve was most common, often associated with an aortic valvar anomaly and/or left ventricular hypertrophy. Patients with the first and second types had more severe involvement than those with the third or fourth types. Patients undergoing transplantation of haematopoietic stem cells seem to stabilize after an initial worsening while, in contrast, we were unable to demonstrate an effect of enzyme replacement therapy on the progression of the cardiac disease, possibly because those receiving such treatment had a higher median age, more severe cardiac disease and shorter follow-up.

Cardiac involvement was present early in more than a half of the patients identified as having mucopolysaccharidosis, and generally progressed, being more frequent and severe in the first and second types of the disease. Longer follow-up is needed to demonstrate any significant improvement induced by new therapies.

Florida is the fourth largest state in the United States of America. In 2004, 218,045 live babies were born in Florida, accounting for approximately 1744 new cases of congenital heart disease. We review the initial experience of The Society of Thoracic Surgeons Congenital Heart Surgery Database with a regional outcomes report, namely the Society of Thoracic Surgeons Florida Regional Report.

Eight centres in Florida provide services for congenital cardiac surgery. The Children’s Medical Services of Florida provide a framework for quality improvement collaboration between centres. All congenital cardiac surgical centres in Florida have voluntarily agreed to submit data to the Society of Thoracic Surgeons Database. The Society of Thoracic Surgeons and Duke Clinical Research Institute prepared a Florida Regional Report to allow detailed regional analysis of outcomes for congenital cardiac surgery.

The report of 2007 from the Society of Thoracic Surgeons Congenital Heart Surgery Database includes details of 61,014 operations performed during the 4 year data harvest window, which extended from 2003 through 2006. Of these operations, 6,385 (10.5%) were performed in Florida. Discharge mortality in the data from Florida overall, and from each Florida site, with 95% confidence intervals, is not different from cumulative data from the entire Society of Thoracic Surgeons Database, both for all patients and for patients stratified by complexity.

A regional consortium of congenital heart surgery centres in Florida under the framework of the Children’s Medical Services has allowed for inter-institutional collaboration with the goal of quality improvement. This experience demonstrates, first, that the database maintained by the Society of Thoracic Surgeons can provide the framework for regional analysis of outcomes, and second, that voluntary regional collaborative efforts permit the pooling of data for such analysis.

To investigate the role of anti-perforin neutralizing antibody in viral myocarditis.

We divided 45 Balb/c mice randomly into 3 groups, a normal control group, a control group inoculated with coxsackie virus B3, and a group inoculated with anti-perforin neutralizing antibody. The second group was inoculated with 0.15 milliliters coxsackie virus B3, and the third group additionally with 0.1milligrams/kilogram anti-perforin neutralizing antibody at time points of 6 hours and 3 days after infection. Histopathology was performed using haematoxylin and eosin, with apoptosis examined by the terminal transferase-mediated 2′-deoxyuridine 5′-triphosphate-biotin nick, end-labeling method, or Tunel. The expression of caspase-3 in myocardium was investigated by immunohistochemistry and reverse-transcription polymerase chain reaction.

The pathologic score, myocardial viral titers, average percentages of apoptotic cardiomyocytes, expression of active caspase-3 protein and messenger ribonucleic acid in the myocardium of the mice receiving anti-PFP neutralizing antibody therapy were all significantly reduced when compared to values from the group inoculated with coxsackie virus B3. The rates of expression of Caspase-3 and myocardial apoptosis were positively correlated with the scores for myocardial pathology.

Our results suggest that anti- perforin neutralizing antibody can reduce the myocardial damage by blocking the perforin/granzyme pathway, and downregulating the expression of messenger ribonucleic acid and protein of Caspase-3. These approaches may offer promising novel therapeutic strategies for the clinical treatment of viral myocarditis.

Multimedia programmes relating to education and consents may be useful for decreasing anxiety during catheter-based repair in patients with congenitally malformed hearts.

Our study was aimed at evaluating the impact of multimedia protocols for education of a population of consecutive patients with congenitally malformed hearts prior to transcatheter repair.

Between September, 2006, and May, 2008, we enrolled 100 consecutive patients, with a mean age of 45 ± 19 years, of whom 69 were female, for catheter-based repair of their congenitally malformed hearts. In the first 50 patients, we used a written form for informed consent sent to the patients 15 days before the procedure, coupled with a personal interview of 30 minutes. In the subsequent 50 patients, we used multimedia protocol for education, comprising a booklet of 4 pages containing a simple and brief explanation of the intervention, and a digital film of 4 minutes showing the transcatheter procedure with a commentary provided by the referring physician, prior to obtaining the signature for informed consent. We then compared the scores for anxiety, the pre-operative heart rate, the frequency of vaso-vagal episodes, and the need for conscious sedation between the two groups.

Patients who underwent preconditioning using the multimedia programme were significantly less anxious, and had significantly lower heart rates. Vaso-vagal episodes were also significantly less in this group, with no episodes compared to 14% in those providing standard informed consent. Conscious sedation was needed more frequently in those providing standard informed consent.

Our brief study suggests that a comprehensive multimedia programme of preparation increases the tolerability, and decrease the emotional state, of adults about to undergo catheter-based interventions for congenital cardiac disease.

There is a high incidence of scoliosis in patients who have undergone cardiothoracic surgery for correction of congenital cardiac disease, this risk being 10 times higher than in the general population.

So as to analyse the surgical and postoperative complications, we designed a retrospective study to include every child who underwent spinal orthopaedic surgery, and who had previously undergone cardiothoracic surgery because of a congenital cardiac malformation. We excluded those patients who had syndromes associated with the development of scoliosis.

We identified 18 patients with surgically treated congenital cardiac disease who had undergone surgery for scoliosis over a period of 7 years. This group came from a total number of 87 patients undergoing spinal fusion over the same period. Of those with congenitally malformed hearts, 61% had acyanotic lesions, with ventricular septal defect being the most frequent single lesion, present in 40%. All the patients needed blood transfusions during the surgery, with aprotinin used in 73% to reduce the bleeding, and inotropes needed for 4 children. During the immediate postoperative period, 1 patient died in the first 24 hours, while 7 (39%) had different complications, pneumonia in 4, pleural effusions in 2, and rhabdomyolysis in the other, as opposed to a rate of complications of 27% in patients without heart disease.

The surgical and postoperative complications in these patients depend on the specific cardiac lesion. A multidisciplinary team with experience in the treatment of congenitally malformed hearts is essential for appropriate management of these patients.

22q11.2 microdeletion is the most common microdeletion in the global population. Congenital cardiac disease is the most frequently observed feature of this syndrome. The prognosis of patients with 22q11.2 copy number aberrations varies from those without 22q11.2 deletion or duplication.

We enrolled 241 patients from Nanjing Drum Tower Hospital and Nanjing Sick Children’s Hospital, 227 being scheduled for cardiac surgery, and 14 cases being fetuses aged from 24 to 36 gestational weeks. We performed karyotypic analysis and multiplex ligation-dependent probe amplification in all cases.

Karyotypic analysis demonstrated 3 cases with trisomy 21, and 1 case with mosaic trisomy 8 [47,XY,+8/46,XY(1:2)]. Multiplex ligation-dependent probe amplification analysis revealed 10 cases (4.15%) with changes in the number of copies within the region of 22q11.2, of which 7 cases were hemizygous interstitial microdeletion from CLTCL1 to LZTR1, 1 case with deletion of the region from CLTCL1 to PCQAP, and 2 cases with 22q11.2 duplication, one of which spanned from ZNF74 to LZTR1, and simultaneously showed trisomy 21 by karyotyping analysis, and the other spanned from HIC2 to TOP3B. The phenotypes of the cardiac lesions included 3 cases of ventricular septal defect, 3 of tetralogy of Fallot, 2 of combined ventricular and atrial septal defects, and 2 with pulmonary arterial stenosis.

Patients with congenitally malformed hearts who are scheduled for cardiac surgery, as well as fetuses with congenital cardiac disease, should routinely undergo karyotypic analysis and examination for 22q11.2 aberrations. Multiplex ligation-dependent probe amplification has been proven to be a cost-effective diagnostic technique for 22q11 deletion syndrome.

Anomalous origin of the left coronary artery from the pulmonary trunk is a rare congenital heart defect. Cardiac catheterization remains the standard means of diagnosis. Our purpose in this study is to emphasize the importance of assessing the electrocardiogram when making the diagnosis, in addition to taking note of transthoracic echocardiographic findings. We also analyzed the sensitivity of each parameter under investigation.

Between June, 1999, and March, 2007, we studied 9 patients, 6 males and 3 females, with a mean age of 3.02 years, in whom anomalous origin of the left coronary artery from the pulmonary trunk was suspected subsequent to transthoracic echocardiographic examination. We examined their electrocardiograms, and undertook cardiac catheterization. In all patients, the transthoracic echocardiogram had shown retrograde flow into the pulmonary trunk, with the left coronary artery arising from pulmonary trunk, along with a dilated right coronary artery, or intercoronary collateral vessels. In 8 patients, the electrocardiogram showed deep Q wave in leads I and aVL, with depression of the ST segments over lead V4 through 6, or inversion of the T waves in leads I, II, and aVL. In the remaining patient, the electrocardiogram showed incomplete right bundle branch block. Later, cardiac catheterization confirmed the diagnosis in 8 patients, but the other patient was shown to have the right coronary artery arising from the pulmonary trunk.

By combining transthoracic echocardiography with study of the electrocardiogram, it is possible to provide accurate evaluation of anomalous origin of the left coronary artery from the pulmonary trunk.