To perform surgical closure of a clinically significant arterial duct on children in a third world country.

An arterial duct is one of the most common congenital cardiac defects. Large arterial ducts can cause significant pulmonary overcirculation, causing symptoms of congestive cardiac failure, ultimately resulting in premature death. Closure of an arterial duct is usually curative, allowing for a normal quality of life and expectancy. In western countries, arterial duct closure in children is usually performed by deployment of a device through a catheter-based approach, replacing previous surgical approaches. In third world countries, there is limited access to the necessary resources for performing catheter-based closure of an arterial duct. Consequently, children with an arterial duct in a third world country may only receive palliative care, can be markedly symptomatic, and often do not survive to adulthood.

We assembled a team of 11 healthcare workers with extensive experience in the medical and surgical management of children with congenital cardiac disease. In all, 21 patients with a history of an arterial duct were screened by performing a comprehensive history, physical, and echocardiogram at the Angkor Hospital for Children in Siem Reap, Cambodia.

A total of 18 children (eight male and ten female), ranging in age from 10 months to 14 years, were deemed suitable to undergo surgery. All patients were symptomatic, and the arterial ducts ranged in size from 4 to 15 millimetres. Surgical closure was performed using two clips, and in four cases with the largest arterial duct, sutures were also placed. All patients had successful closure without any significant complications, and were able to be discharged home within 2 days of surgery. Of note, four children with arterial ducts died in the 5 months before our arrival.

Surgical closure of an arterial duct can be performed safely and effectively by an experienced paediatric cardiothoracic surgical team on children in a third world country. We hope that our experience will inspire others to perform similar missions throughout the world.

Complications, such as secondary hypertension, probably related to the loss of arterial elasticity, frequently arise in Cushing’s syndrome, and may persist even beyond cure. This study aimed at demonstrating that arterial compliance, evaluated by automated recording of the QKd interval, was lower in children after a successful surgery for Cushing’s syndrome than in a control group of healthy subjects.

In all, 23 young girls aged between 11 and 18 years who had undergone a surgical cure for Cushing syndrome – 18 with a pituitary adenoma, three with a primary adrenal disease, and two suffering from ectopic adrenocorticotrope hormone secretion – were enrolled. Arterial stiffness was measured by the standardised non-invasive QKd 100-60 method. A 24-hour ambulatory blood pressure monitoring and a transthoracic echocardiography were also performed.

The children operated for Cushing’s syndrome showed disadvantageous differences in 24-hour ambulatory blood pressure monitoring and in QKD 100-60 value, with p less than 0.01, compared with the control group.

In spite of its successful surgical cure, Cushing’s syndrome results in a significantly decreased arterial distensibility when compared with the control group, which might explain these differences in blood pressure levels. It underlines a significantly higher cardiovascular risk, notwithstanding both the normalisation of cortisol secretion and the very early age of the patients.

Inappropriate heart rate response to exercise – chronotropic incompetence – and exercise intolerance are common in patients with a systemic right ventricle. We aimed to assess the relationship between heart rate increase, oxygen consumption, and timing of the right ventricular cardiac cycle in this cohort.

We prospectively studied nine patients with systemic right ventricles and pre-existing pacemakers using Doppler-echocardiography and treadmill exercise testing. Echocardiography was performed at increasing heart rates. Exercise tests were performed with baseline pacemaker settings and with optimised heart rate response in random order. In addition, eight age- and gender-matched controls underwent exercise testing using a similar exercise protocol.

Patients with a systemic right ventricle had significantly lower peak oxygen consumption compared to controls – 12.6 plus or minus 6.8 versus 31.4 plus or minus 6.6 metres per kilogram per minute (p = 0.0006) – at baseline and active pacemaker reprogramming failed to increase peak oxygen consumption in this cohort – 12.6 plus or minus 6.8 versus 12.4 plus or minus 4.9 millilitres per kilogram per minute (p = NS) at baseline and with reprogramming, respectively. We found not only a marked increase in total isovolumic time but also a significant reduction in total filling time and the aortic velocity time integral, p-value is less than 0.001 for all, at higher heart rates compared to baseline conditions.

This study suggests that despite chronotropic incompetence at baseline, rate-responsive pacing does not improve exercise capacity in patients with a systemic right ventricle. It further indicates that high heart rates may be detrimental in these patients by reducing diastolic filling and stroke volume. These findings may have clinical implications when considering implantation of a permanent pacemaker in this cohort.

The aim of this study was to investigate the relations between the P-wave dispersion and diastolic functions in type 1 diabetic children.

A total of 33 diabetic patients without any cardiovascular disease, with a mean age of 12.3 plus or minus 4.2 years, and 29 healthy controls, with a mean age of 10.4 plus or minus 3.9 years were enrolled for this study. Left and right ventricular functions were assessed by using standard pulsed-wave Doppler echocardiography. P-wave dispersion was calculated by measuring minimum and maximum P-wave duration values on the surface electrocardiogram.

For the diabetic patients, P-wave maximum duration and dispersion was found to be significantly increased compared with healthy controls. Likewise, mitral A velocity and A velocity time integral was significantly increased while the isovolumic contraction time was significantly higher in the diabetics. In tricuspid valve measurements, however, A velocity time integral was found to be significantly higher, whereas the deceleration time was significantly lower in the diabetics. No relation was found between the left ventricle diastolic functions and duration of diabetes, HbA1c levels and P-wave dispersion in the diabetic children. No correlation was found between the diastolic functions and P-wave minimum, maximum duration, and dispersion for all the participants.

In type-1 diabetic children, the diastolic functions of both the ventricles were observed to be affected negatively together. Diabetes might be causing the prolongation of P-wave dispersion, but there was no relationship between the diastolic functions and P-wave dispersion in the diabetic children.

Mucopolysaccharidosis is an inborn error of metabolism causing glucosaminoglycans tissue storage. Cardiovascular involvement is variable but contributes significantly towards the morbidity and mortality of the patients.

To characterise the echocardiographic abnormalities in children and adolescents with different types of mucopolysaccharidosis.

Echocardiograms and medical records of 28 patients aged 2–14 years, seen from 2003 to 2005, were revised. At that time, the enzymatic replacement therapy was still not available in our institution.

Echocardiographic alterations were detected in 26 patients (93%), whereas 16 (57%) had abnormal auscultation, and only 6 (21%) presented with cardiovascular complaint. Mitral valve thickening with dysfunction (regurgitation, stenosis, or double lesion) was diagnosed in 60.8%, left ventricular hypertrophy in 43% and aortic valve thickening with regurgitation in 35.8% of the patients. There was no systolic dysfunction and mild left diastolic dysfunction was shown in 21.5% of the patients. Pulmonary hypertension was present in 36% of the patients, causing the only two deaths recorded. There was a strong association between the accumulation of dermatan sulphate and the presence of mitral valve dysfunction (p = 0.0003), aortic valve dysfunction (p = 0.006), and pulmonary hypertension (p = 0.006). Among individuals with two or more examinations, 82% had a worsening evolution.

Echocardiographic alterations in children with Mucopolysaccharidosis are frequent and have a progressive character. Left valve lesions, ventricular hypertrophy, and pulmonary hypertension were the most common findings and there was an association between the accumulation of dermatan sulphate and cardiovascular involvement. Unlike in adults, pulmonary hypertension was the main cause of death, not left ventricle systolic dysfunction.

β-blockers improve the prognosis of patients with cardiac failure due to left ventricular systolic dysfunction. The aim of this study was to assess the efficacy of β-blockers in patients with dysfunctional systemic right ventricle.

Fourteen patients with systemic right ventricle following a Mustard or a Senning operation for the transposition of the great arteries, or congenitally corrected transposition were included in the study. All had a decreased systemic right ventricular ejection fraction despite having standard cardiac failure therapy. Quality of life, New York Heart Association class, aerobic capacity, and systemic right ventricular function were assessed before treatment with β-blockers and at the end of the follow-up period, mean of 12.8 months with a range from 3 to 36 months.

Change in New York Heart Association class was significant (p = 0.016). Quality of life improved significantly throughout the study from a median grade 2 with a range from 1 to 3 to a median grade 1 with a range from 1 to 2 (p = 0.008). Systemic right ventricular ejection fraction assessed by radionuclide ventriculography improved significantly from a median of 41% (range: 29–53%) to 49% (range: 29–62%; p = 0.031). However, the change in thee ejection fraction assessed by magnetic resonance imaging was not significant from a median of 29% (range: 12–47%) to 32% (range: 22–63%; p = 0.063).

In patients with cardiac failure due to systemic right ventricular dysfunction, β-blockers improve New York Heart Association class, quality of life, and systemic right ventricular ejection fraction assessed by radionuclide ventriculography.

We aimed to determine whether N-terminal pro-brain natriuretic peptide can differentiate between cardiac and pulmonary aetiologies of dyspnoea, if N-terminal pro-brain natriuretic peptide can be used for evaluating the effect of treatment in cardiac failure, and for predicting severe pulmonary diseases that are complicated by cardiac failure.

In all, 76 children with dyspnoea were enrolled; 41 of them suffered cardiac failure – 25 caused by cardiac disease, 16 caused by pulmonary disease – and 35 had dyspnoea due to pulmonary disease. The control group consisted of 32 children. We calculated Ross scores, analysed N-terminal pro-brain natriuretic peptide levels, and evaluated left ventricular systolic functions by echocardiography.

N-terminal pro-brain natriuretic peptide levels were significantly higher in children with cardiac failure than in those with pulmonary disease and in controls (medians 7321, 241, 87.71 picograms per millilitre, respectively), were higher in children with cardiac failure due to pulmonary disease than in those with only pulmonary disease (medians 2728, 241 picograms per millilitre, respectively), and were higher in children who died from cardiac failure than in survivors (p < 0.05). After treatment of cardiac failure, N-terminal pro-brain natriuretic peptide levels decreased significantly (p < 0.001). The cut-off level of N-terminal pro-brain natriuretic peptide for differentiating cardiacfailure from pulmonary disease was 726.8 picograms per millilitre, sensitivity 100%, specificity 94.3%.

N-terminal pro-brain natriuretic peptide levels can differentiate dyspnoea due to cardiac failure from pulmonary diseases. It can also be used to monitor the effects of treatment of cardiac failure and to estimate the prognosis, as well as to predict pulmonary diseases that are complicated with cardiac failure.

Thromboembolic events are a serious complication occurring in critically ill children admitted to the cardiac intensive care unit. Although enoxaparin is one of the current anticoagulants of choice, dosages in children are extrapolated from adult guidelines. Recent data suggest that this population may need a higher dose than what is currently recommended to achieve target anti-factor Xa levels. The purpose of this study was to evaluate whether children less than 2 years old admitted to the cardiac intensive care unit require a higher enoxaparin dose than that currently recommended to achieve target anti-factor Xa levels.

Retrospective chart review including patients who received enoxaparin for the treatment or prophylaxis of venous thrombosis between January, 2005 and October, 2007. Patients were classified as younger and older as well as prophylactic and therapeutic on the basis of age and enoxaparin dose, respectively. Younger patients were those 2 month old or less and older patients were those older than 2 months of age.

A total of 31 patients were identified; 13 (42%) were 2 months or younger and 25 (81%) were postoperative patients. Ten (32%) received prophylactic and 21 (68%) received therapeutic enoxaparin doses. To achieve optimal anti-factor Xa levels, enoxaparin dose was increased in all groups and reached statistical significance in all patients except those older than 2 months who received prophylactic enoxaparin. An average of 2.8 dosage adjustments was needed. No bleeding complications were reported.

Young children, infants, and neonates admitted to the cardiac intensive care unit required a significantly higher enoxaparin dose than that currently recommended to achieve target anti-factor Xa levels.

Rashkind balloon atrial septostomy is a common cardiac procedure aimed at improving systemic oxygenation in newborns with cyanotic congenital cardiac defects, such as transposition of the great arteries. Recent reports on the safety of this procedure were from limited series at single institutions. We analysed two complementary national databases to evaluate clinically relevant outcomes of this procedure.

We performed an analysis of transposition of the great artery patients nationwide using 15 years of the Nationwide In-patient Sample and three complementary years of the Kids’ Inpatient Database. Variables included gender, race, age, and co-existing diagnoses. Outcomes included mortality, length of stay, and hospital charges. Comparison between patients undergoing Rashkind procedure or not was performed using Pearson’s chi-square and Kruskal–Wallis tests. We identified 8681 patients with transposition of the great arteries, of whom 1742 (20%) underwent Rashkind procedure. Patients undergoing Rashkind procedure had lower mortality (10% versus 12%, p = 0.021), despite higher median co-morbidities and longer median length of stay. Rashkind procedure was not associated with increased risk of necrotising enterocolitis (1% versus 1%, p = 0.630), but was associated with nearly twice the risk of clinically recognised stroke (1% versus 0%, p = 0.046).

This study represents the largest national analysis of transposition of the great artery patients to date, with a subset treated with Rashkind procedure. Patients not undergoing Rashkind procedure had higher mortality. Rashkind procedure was not associated with increased risk of necrotising enterocolitis, but was associated with twice the risk of stroke.

To investigate the utility of computed tomographic angiography as an adjunctive imaging modality before congenital cardiac surgery.

We evaluated 33 patients who underwent a pre-operative computed tomographic angiogram. They were classified according to the anatomic site of repair. Post-operatively, the surgeon completed a questionnaire assessing the utility of the study.

Computed tomographic angiography was found to be either “essential” or “very useful” for pre-operative planning in 94% of the patients. Specifically, the scan was consistently useful for procedures involving the aorta (14/15, 93%) or the pulmonary veins (4/4, 100%) and obviated pre-operative catheterisations in 14 patients (42%). Furthermore, when compared with other diagnostic groups, computed tomographic angiography determined the need for peripheral cannulation in patients undergoing re-operations (6/7; 86%, p = 0.02).

Computed tomographic angiography was found to be useful in the pre-operative planning of virtually all patients undergoing repair of congenital cardiac malformations, regardless of diagnosis. Specifically, the studies were essential in select populations, such aortic arch or pulmonary vein repairs, and helped to determine cannulation sites for repeat operations while significantly reducing the need for invasive imaging.

CD34 positive, KDR/CD34, CD133/CD34, and CD117/CD34 double positive progenitor cells were measured in 24 obese children and adolescents – 15 female; age: 12.5 plus or minus 2.1 years, body mass index standard deviation score: 2.5 plus or minus 0.5, waist: 88.6 plus or minus 15.0 centimetre, body fat: 24.6 plus or minus 2.2% – participating in the CHILT III programme. Percentage body fat was assessed by skinfold thickness. Peak of oxygen uptake and the respiratory quotient were determined by spiroergometry.

No gender differences were found. CD34 positive and CD117 positive/CD34 positive cells correlated with maximum relative watt performance, r is equal to 0.429 and 0.462; p-value less than 0.05. The peak of oxygen uptake correlated with CD34 positive and CD133 positive/CD34 positive cells, r is equal to 0.458 and 0.456; p-value less than 0.05, while no correlations were found between parameters of weight, body composition, and respiratory quotient with progenitor cells.

A higher physical fitness, but not less body fat or body mass index is associated with a higher number of endothelial progenitor cells. These results support the hypothesis that physical fitness and cardiovascular risk in high-risk populations are inversely related. Further research is warranted to clarify the strength of this association and longitudinal effects of a comprehensive obesity programme.

Usually the literature results for device-closure of patent oval foramen concern a single type of device or different devices implanted without anatomical preferences. We propose a strategy of device type and selection based on intra-cardiac echocardiography measurements of inter-atrial septum characteristics.

We prospectively enrolled 100 consecutive patients with amean age of 43 plus or minus 15.5 years, 68 females, who had been referred to our centre for catheter-based closure of inter-atrial shunts over a 48-month period. On the basis of intra-cardiac echocardiography findings the operators selected the Amplatzer Occluder family (AGA Medical Corporation) or the Premere Closure System (St Jude Medical Inc.). Determinants of the selection process were presence and extension of atrial septal aneurysm, tunnel length, rims length, and thickness, presence of additional fenestrations.

According on intra-cardiac echocardiography study, 26 patients have a long channel patent oval foramen, 44 patients had a large atrial septal aneurysm (more than four RL), 24 patients had a moderate atrial septal aneurysm (more than two RL but less than four right-to-left), and six patients had hypertrophic rims. Thus, the Amplatzer PFO Occluder was selected in 24 patients, the Amplatzer ASD Cribriform Occluder in 44 patients, and the Premere device in 32 cases. No aortic erosions, device thrombosis, or recurrent ischaemic cerebral events were observed. Pre-discharge and follow-up occlusion rates were 91% and 96%, respectively.

Our study suggested that such strategy driven from identification and measurements of the right atrium and inter-atrial septum components resulted in low complications and high-success rates, mandatory conditions when facing with otherwise healthy subjects, such as the patients with patent oval foramen.