To determine the safety and efficacy of the Amplatzer duct occluder and the Amplatzer duct occluder II in different types of arterial ducts, and to determine in which types of ducts the use of this new device can be advantageous.

All children with a device-based ductal closure between September, 2005 and February, 2010 were included. We retrospectively analysed the catheterisation and follow-up data.

Between September, 2005 and February, 2010, 44 ducts were closed with the Amplatzer duct occluder – group Amplatzer duct occluder – and 52 ducts were closed with the Amplatzer duct occluder II – group Amplatzer duct occluder II. In the Amplatzer duct occluder group, the mean age was 3 years and 4 months, and the mean weight was 14.7 kilograms. Closure was successfully performed in all children. Complete closure at 24 hours was attained in 42 of 44 children (95.45%). No major complications occurred. In the Amplatzer duct occluder II group, the mean age was 6 years and 2 months, and the mean weight was 25.4 kilograms. Closure was successfully performed in all children, except in two children in whom the occluder protruded into the aortic isthmus and was replaced by the Amplatzer duct occluder. Complete closure at 24 hours was attained in 51 of 52 children (98.08%). No major complications occurred.

In our experience, duct closure with the Amplatzer duct occluder II is a safe and effective method. The advantages of using it are the smaller sheath sizes and softer shape.

Ellis – van Creveld syndrome is an autosomal recessive disorder manifest by short-limb dwarfism, thoracic dystrophy, postaxial polydactyly, dysplastic nails and teeth, and an approximately 60% incidence of congenital malformations of the heart. Despite patients with Ellis – van Creveld syndrome being regarded as having a high surgical risk, few data are available regarding their outcomes following surgery for congenital malformations of the heart in the current era.

In this retrospective report, we summarise the clinical observations and outcomes of nine infants with Ellis – van Creveld syndrome who underwent surgery for congenital malformations of the heart between 2004 and 2009.

We identified 15 patients with Ellis – van Creveld syndrome during the study period; 11 (73%) had haemodynamically significant congenital malformations of the heart warranting surgery. In two of these patients, surgery was not performed. Of the nine patients who underwent surgery, all of whom were infants, eight (89%) had various forms of an atrioventricular septal defect and one patient (11%) had hypoplastic left heart syndrome (mitral and aortic atresia). Among the nine patients who underwent surgery, four (44%) died at a median of 102 days with a range of 25–149 days post-operatively, mostly from respiratory failure. Respiratory morbidity was seen in all surviving patients, of whom three underwent tracheostomy.

Surgery for congenital malformations of the heart can be successful in infants with Ellis – van Creveld syndrome, but mortality is high and post-operative respiratory morbidity should be expected.

Owing to systemic inflammatory response syndrome, the diagnosis of post-operative infection after cardiopulmonary bypass is difficult to assess in children with the usual clinical and biological tools. Procalcitonin could be informative in this context.

Retrospective study in a paediatric intensive care unit. Blood samples were collected as soon as infection was clinically suspected and a second assay was performed 24 hours later. Using referenced criteria, children were retrospectively classified into two groups: infected and non-infected.

Out of the 95 children included, 14 were infected. Before the third post-operative day, procalcitonin median concentration was significantly higher in the infected group than in the non-infected group – 20.24 nanograms per millilitre with a 25th and 75th interquartile of 15.52–35.71 versus 0.72 nanograms per millilitre with a 25th and 75th interquartile of 0.28 to 5.44 (p = 0.008). The area under the receiver operating characteristic curve was 0.89 with 95% confidence intervals from 0.80 to 0.97. The best cut-off value to differentiate infected children from healthy children was 13 nanograms per millilitre with 100% sensitivity – 95% confidence intervals from 51 to 100 – and 85% specificity – 95% confidence intervals from 72 to 91. After the third post-operative day, procalcitonin was not significantly higher in infected children – 2 nanograms per millilitre with a 25th and 75th interquartile of 0.18 to 12.42 versus 0.37 nanograms per millilitre with a 25th and 75th interquartile of 0.24 to 1.32 (p = 0.26). The area under the receiver operating characteristic curve was 0.62 with 95% confidence intervals from 0.47 to 0.77. A procalcitonin value of 0.38 nanograms per millilitre provided a sensitivity of 70% with 95% confidence intervals from 39 to 89 for a specificity of 52% with 95% confidence intervals from 34 to 68. After the third post-operative day, a second assay at a 24-hour interval can improve the sensitivity of the test.

Procalcitonin seems to be a discriminating marker of bacterial infection during the post-operative days following cardiopulmonary bypass in children.

To analyse the relative frequency and gender ratios in the procedures (both surgical and catheter-based interventions) performed for cardiac diseases in Indian children.

Not many studies are available in the developing countries with regard to the prevalence, relative frequency, or gender distribution of cardiac diseases in children. As universal newborn screening is not carried out for congenital cardiac diseases, the statistics are difficult to ascertain. Do female and male children with cardiac disease get equal parental preference in terms of surgical correction and catheter interventions in India? This question is also unanswered.

Analysis of 15,066 consecutive Indian children aged <18 years who were operated upon or had catheter intervention at a single tertiary care centre. Relative frequency and male/female ratios of cardiac lesions in these children were determined and compared with the studies in literature.

Overall male/female ratio in the study was 1.4:1. Ventricular septal defect was the most common lesion (24.2%) with male/female ratio of 1.5:1, followed by tetralogy of Fallot (18.7%, 1.6:1), atrial septal defect (14.4%, 0.9:1) and so on. Male children dominated the total number of procedures performed.

In most of the individual cardiac lesions, the relative frequency was different from that of international studies. The gender ratio for the majority of the individual type of cardiac problems was different from that of international references. These findings may suggest a preference for the male child in the treatment of cardiac diseases in India, which could possibly be related to social causes.

Differentiation of the so-called sinus venosus defect from other defects permitting shunting between the atrial chambers remains problematic. The lesion is not a true septal defect, and current theories to explain the existence of the sinus venosus defect fall short. The presence of persistent systemic to pulmonary venous connections has been proposed to explain the existence of the sinus venosus defect.

Clinical histories and radiological findings of six patients are reviewed. Three patients have veno-venous bridges, two have partial anomalous pulmonary venous connections, and one patient has a sinus venosus defect. The clinical information is reviewed, along with current developmental and morphological considerations.

We provide radiographic, developmental, and morphological evidence to support the theory that a so-called sinus venosus defect is the consequence of persistence of foetal systemic to pulmonary veno-venous bridges, rather than of deficiencies in atrial septation.

The aim of this study is to evaluate the cumulative detection rate of foetal echocardiography during gestation and in the early neonatal period, with a special emphasis on early foetal echocardiography.

We conducted a retrospective survey of all singleton pregnancies from 1993 to 2007, with complete sequential echocardiography from 11 plus 0 to 13 plus 6 weeks of gestation. It was mandatory to have at least one foetal echocardiography in the second or third trimester and one postnatally.

Our study included 3521 pregnancies, in which 77 cases were diagnosed with congenital heart disease. Of them, 66 were detected in the first trimester – 11 plus 0 to 11 plus 6 weeks: 22 cases; 12 plus 0 to 12 plus 6 weeks: 23 cases; 13 plus 0 to 13 plus 6 weeks: 21 cases – with an 85.7% detection rate of congenital heart disease in early foetal echocardiography. In the second trimester, seven cases were found, with a detection rate of 9.1%. The third trimester reported two cases, with a detection rate of 2.6%. Postnatally, two (2.6%) cases were detected. The overall in utero detection rate of congenital heart disease was 97.4%.

Foetal echocardiography performed at the time of anomaly screening in the first trimester results in high detection rates of congenital heart disease. Cardiac pathology may evolve, and further examinations at later stages of pregnancy could improve the detection rate of congenital heart disease.

Remote ischaemic pre-conditioning and cardiac ischaemic post-conditioning provide myocardial protection in cardiac surgery. However, these two endogenous strategies have not been directly compared in a clinical setting. The purpose of this study was to compare the efficacy of remote ischaemic pre-conditioning and post-conditioning in providing myocardial protection to children undergoing cardiopulmonary bypass for surgical repair of ventricular septal defect.

We randomly assigned 60 paediatric patients scheduled for surgical correction of congenital ventricular septal defect to the post-conditioning group (n = 20), remote pre-conditioning group (n = 20), or control group (n = 20). Post-conditioning consisted of 30 seconds of ischaemia and 30 seconds of reperfusion achieved by clamping and unclamping the aorta, repeated three times over 3 minutes immediately after cardioplegic arrest. Remote ischaemic pre-conditioning consisted of 5 minutes of lower limb ischaemia followed by 5 minutes of reperfusion using a blood-pressure cuff inflated to a pressure of 200 millimetres of mercury, also repeated three times over 30 minutes. We assayed creatine kinase-MB, troponin I.

Mean age, cardiopulmonary bypass times, and aortic cross-clamp times were matched across groups. Both post-conditioning and remote ischaemic pre-conditioning reduced the peak release of creatine kinase-MB (86.1 plus or minus 24.1 units per litre and 92.8 plus or minus 20.6 units per litre, respectively, versus 111.0 plus or minus 44.6 units per litre in the control, p less than 0.05) and troponin I (0.28 plus or minus 0.10 nanogram per millilitre and 0.26 plus or minus 0.09 nanogram per millilitre, respectively, versus 0.49 plus or minus 0.19 nanogram per millilitre in the control group, p less than 0.05).

Our study demonstrates that ischaemic post-conditioning and remote ischaemic pre-conditioning provide comparable myocardial benefit in children undergoing cold blood cardioplegic arrest.

QTc prolongation has been reported in adults following cardiopulmonary bypass; however, this phenomenon has not been studied in children with congenital cardiac disease. This study's aim was to formally assess QTc in children undergoing cardiac surgery.

Pre-operative and post-operative electrocardiograms during hospital stays were prospectively analysed on 107 consecutive patients under 18 years of age undergoing cardiac surgery. QTc was measured manually in leads II, V4, and V5. Measurements of 440 and 480 milliseconds were used to categorise patients. Peri-procedural data included bypass and cross-clamp time, medications, and electrolyte measurements. Outcome data included arrhythmias, length of mechanical ventilation, and hospital stay. Patients with post-operative new bundle branch block or ventricularly paced rhythm were excluded.

In all, 59 children were included, out of which 26 had new QTc over 440 milliseconds and 6 of 59 had new QTc over 480 milliseconds post-operatively. The mean increase in post-operative QTc was 25 milliseconds, p=0.0001. QTc over 480 was associated with longer cross-clamp time, p=0.003. Other risk factors were not associated with post-operative QTc prolongation. This phenomenon was transient with normalisation occurring in 67% of patients over 60 hours on average. One patient with post-operative QTc over 440 milliseconds developed ventricular tachycardia. There was no correlation between prolonged QTc and duration of mechanical ventilation, or hospital stay.

A significant number of children undergoing cardiac surgery showed transient QTc prolongation. The precise aetiology of QT prolongation was not discerned, though new QTc over 480 milliseconds was associated with longer cross-clamp time. In this cohort, transient QTc prolongation was not associated with adverse sequela.

To describe the difficulties and differing techniques in the transcatheter placement of amplatz ventricular septal defect devices to close perimembranous ventricular septal defects and place these in the context of the expanding literature on ventricular septal defect catheter closure.

Surgery remains the established first-line therapy for closure of haemodynamically significant perimembranous ventricular septal defects. Transcatheter techniques appeared to promise a possible alternative, obviating the need for cardiac surgery. However, significant technical and anatomical constraints coupled with ongoing reports of a high incidence of heart block have prevented these hopes from being realised to any significant extent. It is likely that there are important methodological reasons for the high complication rates observed. The potential advantages of transcatheter perimembranous ventricular septal defect closure over surgery warrant further exploration of differing transcatheter techniques.

Between August, 2004 and November, 2009, 21 patients had a perimembranous ventricular septal defect closed with transcatheter techniques. Of these, 14 were closed with a muscular amplatz ventricular septal defect device. The median age and weight at device placement were 8 years, ranging from 2 to 19 years, and 18.6 kilograms, ranging from 10 to 21 kilograms, respectively.

There were 25 procedures performed on 23 patients using 21 amplatz ventricular septal defect devices. Median defect size on angiography was 7.8 millimetres, ranging from 4 to 14.3 millimetres, with a median device size of 8 millimetres, ranging from 4 to 18 millimetres, and a defect/device ratio of 1.1, with a range from 0.85 to 1.33. Median procedure time was 100 minutes, with a range from 38 to 235 minutes. Adverse events included device embolisation following haemolysis in one, and new aortic incompetence in another, but there were no cases of heart block. Median follow-up was 41.7 months, with a rangefrom 2 to 71 months.

Evaluating transcatheter closure of perimembranous ventricular septal defect using amplatz ventricular septal defect devices remains important, if a technically feasible method with low and acceptable complication rates is to be identified. Incidence of heart block may be minimised by avoiding oversized devices, using muscular devices, and accepting defeat if an appropriately selected device pulls through. Given the current transcatheter technologies, the closure of perimembranous ventricular septal defects should generally be performed in children when they weigh at least 10 kilograms.

In developing countries, congenital heart disease is often unrecognised, leading to serious morbidity and mortality. Guatemala is one of the few developing countries where expert paediatric cardiac treatment is available and affordable, and therefore early detection could significantly improve outcome. We assessed regional congenital heart disease detection rates in Guatemala, and determined whether they correlated with the regional human development index.

We retrospectively reviewed all new cardiac referrals made in 2006 to the Unidad de Cirugia Cardiovascular Pediatrica, the only paediatric cardiac centre in Guatemala. We calculated regional detection rates by comparing the number of congenital heart disease referrals with the expected incidence using the National Ministry of Health birth data. We then compared the regional detection rates with the human development index data published in the United Nations 2006 Development Program Report using Spearman’s rank correlation.

An estimated 3935 infants with cardiac defects were born in Guatemala in 2006, an expected 1380 (35%) of whom had severe forms. Overall, only 533 children (14%) with cardiac defects were referred. Of these, 62% had simple shunt lesions, 13% had cyanotic lesions, and 10% had left-sided obstructive lesions. Only 11.5% of referred patients were neonates. Regional detection rates, ranged 3.2–34%, correlated with the regional human development index (r = 0.75, p < 0.0001).

Current detection of congenital heart disease in Guatemala is low and correlates with the regional human development index. Those detected are older and have less severe forms, suggesting a high mortality rate among Guatemalan neonates with complex cardiac defects.

This study evaluates the efficacy and safety of sildenafil in patients with Eisenmenger's syndrome with special emphasis on haemodynamic parameters and its comparative efficacy in atrial septal defect versus ventricular septal defect patients.

Oral sildenafil was given to 22 patients with Eisenmenger's syndrome – eight with atrial septal defect and 14 with ventricular septal defect – after detailed baseline evaluation including a six-minute walk test, echocardiography, and cardiac catheterisation. Patients were followed up for a period of 6 months for functional class assessment and six-minute walk distance. Cardiac catheterisation was repeated in all patients.

A significant improvement in the World Health Organization functional class, six-minute walk distance, mean pulmonary arterial pressure, and pulmonary vascular resistance was noticed. Systemic arterial and mixed venous oxygen saturations were also significantly improved along with improvement in pulmonary blood flow. None showed any significant side effects or worsening of systemic arterial saturation. At baseline, mean pulmonary arterial pressure, pulmonary vascular resistance, and pulmonary/systemic vascular resistance ratios were significantly higher in ventricular septal defect patients than in atrial septal defect patients. Atrial septal defect patients showed better response in clinical as well as haemodynamic parameters.

Sildenafil is an effective and safe agent for patients with Eisenmenger's syndrome. It improves their functional capacity as well as haemodynamic parameters. The beneficial effects are greater in patients with Eisenmenger's syndrome secondary to atrial septal defect than ventricular septal defect.

In patients with varying degrees of left heart hypoplasia, it is often difficult to determine whether the left heart structures are adequate in size to support biventricular circulation. Historically, the decision to pursue a single ventricle or biventricular repair needed to be made early and was often irreversible. The hybrid procedure may be a better initial approach for patients with borderline left ventricles.

We describe a series of four patients with various congenital cardiac malformations, all of whom had borderline left ventricles. Based on pre-operative echocardiograms, several scoring systems and left ventricle volumes were used to predict the optimal type of repair. A left ventricular volume of 20 millilitres per square metre was used as the minimum cut-off value for adequacy of biventricular repair.

The left ventricular volumes for the patients were 17.1, 23.7, 25.4, and 25.8 millilitres per square metre. In none of the four patients were the calculations unanimous in the recommendation to pursue either type of repair. All patients underwent the hybrid procedure and then eventual single ventricle palliation (two patients) or biventricular repair (two patients). All survived with a mean follow-up of 18 plus or minus 3.9 months.

The hybrid procedure may be the best option in patients with a borderline left ventricle. It can serve as a bridge to a more definitive repair when patients are older, larger, and for whom the decision between single ventricle and biventricular repair can be more easily made.