Electrical injury can result in a variety of cardiac abnormalities. We evaluate the cardiac effects in patients injured by electric shock and treated in our medical centre.

We reviewed retrospectively the findings in 52 children, aged from 7 months to 17 years, with a mean age of 10.1 ± 5.1 years, all evaluated and treated for accidental electric shock from January, 1992, through July, 2004. Relevant data regarding clinical presentation, electrocardiogram recording and cardiac enzymes was compiled. We also evaluated the echocardiographic findings, clinical course, treatment, and outcome.

Syncope had been the presenting symptom in 17 children (33%), asystole in 1 patient, and ventricular fibrillation or tachycardia in 2 patients. Characteristic changes of acute ischaemia of the anterior wall on the basis of changes in the ST segments were noted in 2 patients. Total creatine phosphokinase was measured in 33 children (63%), and was elevated in 20. Creatine phosphokinase-MB was measured in 11 patients, and was abnormal in six (54%). Troponin was measured in three children, and was significantly high in one (33%). Cardiopulmonary resuscitation and mechanical ventilation for a significant period was necessary in 5 patients, of whom 4 (80%) survived. None of the survivors was left with any cardiac disability following the acute event.

Significant cardiac damage and complications are rare in children and young adults who survive incidental electrocution. Most of the cardiac events are observed during the acute phase and immediately subsequent to electrocution. No delayed complications are anticipated.

Anomalous origin of the left coronary artery from the pulmonary trunk is rare, occurring at an incidence of 1 in 300 000. If not diagnosed and treated early, it is life-threatening. Children with the anomaly usually present in infancy with congestive cardiac failure, and are occasionally referred for cardiac transplant. We investigated the medium term outcome for patients following creation of a two-coronary arterial circulation.

Between 1992 and 2007, we diagnosed 15 patients seen at our Institution as having anomalous origin of the left coronary artery from the pulmonary trunk. Over a period of 13 years, aortic reimplantation was undertaken in 12 of these patients, who form the studied cohort.

Direct reimplantation was performed in 5 patients. In 3 cases, a tension-free anastomosis was created using a caudally based flap. In another 3 cases, an extended flap was used, while a patch arterioplasty was fashioned in the final patient. There were no deaths. Left ventricular function recovered in all but one of the patients, and all patients had a reduction in the degree of mitral regurgitation.

Among the variety of surgical techniques, transfer of the anomalous left coronary artery to the aorta is the ideal method for long-term patency and adequate blood supply. This can be achieved by creating flaps based on the walls of the pulmonary trunk and aorta, producing a dual coronary arterial supply with no mortality and low morbidity.

It is accepted treatment to give vasopressin to adults in postcardiotomy shock, but such use in children is controversial. Cardiopulmonary bypass is presumed to attenuate the normal endogenous vasopressin response to shock. We hypothesized that levels of vasopressin in children are altered by bypass, and that children having low endogenous levels perioperatively are more likely to develop hypotension, or require vasopressors.

Serial levels of vasopressin were assessed prospectively in children undergoing bypass at a single center.

Of 61 eligible patients, we enrolled 39 (63%). Their median age was 5 months. The mean level of vasopressin prior to bypass was 18.6 picograms per millilitre, with an interquartile range from 2.6 to 11.4. Levels of vasopressin peaked during bypass at 87.1, this being highly significant compared to baseline (p < 0.00005), remained high for 12 hours at a mean of 73.5, again significantly different from baseline (p = 0.002), were falling at 24 hours, with a mean of 28.1 (p = 0.04), and had returned to baseline by 48 hours, when the mean was 7.4 (p = 0.3). Age, gender, and the category for surgical risk had no influence on the levels of vasopressin. There was no statistically significant relationship between the measured levels and hypotension or the requirement for vasopressors, although a few persistently hypotensive patients had high levels subsequent to bypass. Higher levels correlated with higher levels of sodium in the serum (rs = 0.37, p < 0.00005), higher osmolality (rs = 0.37, p < 0.00005), and positive fluid balance (rs = 0.23, p < 0.008). Preoperative use of inhibitors of angiotensin converting enzyme, preoperative congestive cardiac failure, and longer periods of bypass predicted higher levels during the first eight postoperative hours.

Children do not have deficient endogenous levels of vasopressin following bypass, and lower levels are not associated with hypotension. Any therapeutic efficacy of infusion of vasopressin for post-cardiotomy shock in children is likely due to reasons other than physiologic replacement.

The mitral valvar complex is difficult to visualise accurately in only two dimensions. Three-dimensional echocardiography gives new insight into the dynamic changes of intra-cardiac structures during the cardiac cycle. The aim of this study was to study the mitral annulus in systole and diastole in normal children using three-dimensional echocardiography, and to analyse the effect of regurgitation on annular function.

Three-dimensional echocardiographic datasets, acquired in 11 consecutive subjects with mitral regurgitation, and 20 normal subjects, were analysed offline using simultaneous multiplanar review.

The mitral valvar annular area decreased in diastole, and increased in systole, in both groups. The annulus in patients with mitral regurgitation is dilated compared to normal subjects, the systolic value for those with regurgitation having a mean of 6.79 plus or minus 2.55 centimetres2/metres2, and the diastolic value a mean of 5.01 plus or minus 1.78 centimetres2/metres2, as opposed to a systolic mean value of 5.28 centimetres2/metres2 plus or minus 1.68, p = 0.091, and diastolic mean value of 3.05 centimetres2/metres2 plus or minus 0.90, in normal subjects (p less than 0.0001). The proportional change in mitral valvar annular area from systole to diastole showed a trend towards being smaller in those with mitral regurgitation, although this did not reach significance (24.8% versus 41.13%, p equal to 0.249). Analysis of subgroups of patients with moderate or severe mitral regurgitation showed mitral excursion, expressed as percentage of left ventricular length, to be significantly less than in normal subjects, at 12.78 plus or minus 5.10% versus 15.84 plus or minus 4.23% (p equal to 0.012).

Mitral valvar annular area in children decreases in diastole, and increases in systole. In those with mitral regurgitation, the annulus is dilated and the dynamic annular function is depressed.

To maintain pulmonary valvar function subsequent to repair of tetralogy of Fallot, we have inserted a homograft monocusp when a transjunctional patch was required. In this study, we have evaluated the mid- to long-term outcomes, aiming to determine the durability of the homograft.

Among 218 repairs performed for tetralogy of Fallot between July, 1996, and June, 2005, we inserted homograft monocusps in 54 patients, 4 of whom had associated absent pulmonary valve syndrome, 3 had pulmonary valvar atresia, and 1 had an atrioventricular septal defect with common atrioventricular junction. The median body weight at surgery was 7.8 kilograms, with a range from 3.9 to 42 kilograms. The function of the monocusp valve was assessed by regular echocardiography, using the Kaplan-Meier method and the Cox regression model for statistical analyses.

There were 2 early deaths (3.7%), associated with respiratory infection. No late deaths were observed during the follow-up, which ranged from 0.3 to 120 months, with a median of 64.3 months. Freedom from valvar dysfunction was 67.2 ± 6.7% at 1 year, 37.1 ± 7.3% at 3 years, 23.8 ± 6.7% at 5 years, and 21.2 ± 6.4% at 7 years. We needed to replace the valve in 1 patient during follow-up. We found that ABO blood group incompatibility, stenosis of the pulmonary arteries, and associated absent pulmonary valve syndrome all adversely affected the function of the monocusp.

Our experiences show that insertion of a homograft monocusp can prevent pulmonary regurgitation in the early period after repair of tetralogy of Fallot, but the effects are limited in duration as degeneration progressed. We still need to determine whether this finding can improve the longer-term function of the right ventricle.

With improvements in the surgical and medical management of children with congenital heart disease, the majority are now expected to transition into adult services. Whilst the importance of successful transition is increasingly acknowledged, previous research has identified a number of areas of concern. We therefore wished to elicit the views of young people themselves concerning their requirements from a specialist cardiac service.

Using an exploratory approach our aims were to increase awareness of the service needs and expectations of young people, identify good practice and areas where communication processes and facilities could be improved, and enhance understanding of transition between paediatric and adult services. Postal questionnaires were sent to 63 young people with congenital cardiac disease aged 17 to 20 years. Open ended questions asked for views and experiences about facilities in the paediatric and adult areas, perceptions of transition between services and suggestions for improvements. Qualitative data were transferred verbatim into electronic format to facilitate coding and theming.

Thirty-eight (60.3 percent) completed questionnaires were received. A number of themes, including communication, care processes/caring interactions and environment, emerged regarding areas of satisfaction and dissatisfaction within the paediatric and adult services, together with areas for improvement and important aspects of transition.

Young people’s views need to be taken into account at all stages of the patient journey. They want an individualised approach to their care which recognises that they have specific emotional and practical needs. Such findings need to be incorporated into an ongoing model of care.

By means of retrospective analysis of our institutional experience, we reviewed the clinical manifestation and outcomes of patients subsequent to surgical repair of double-chambered right ventricle.

Between 1988 and 2005, we performed surgical repair in 35 of 37 patients diagnosed with double-chambered right ventricle. The patients ranged in age from 4 to 69 years, with a mean of 21.3 years. Most presented in infancy, with initial manifestation of a short systolic murmur in 34 (92%) of all cases. Pressure gradients were measured invasively across the right ventricular outflow tract of between 30 and 140 mmHg, with a median of 60 mmHg. An associated ventricular septal defect was present in 26 patients (70%). Of the group, 4 patients were aged over 40 years, and 2 had previously undergone operative closure of a ventricular septal defect.

The operative interval ranged from 2 months to 41 years, with a median of 9 years. In all, we resected muscular bundles and enlarged the right ventricular outflow tract. There was no hospital or late death. Median follow-up subsequent to surgery was 7 years, with a range from 0.4 to 11 years. No patient required further surgery to relieve any obstruction of the right ventricular outflow tract, nor long term medical therapy or pacing because of cardiac arrhythmia.

Surgical repair of a double-chambered right ventricle yields excellent haemodynamic and functional results over the mid to long term.

Establishing stable and adequate flow of blood to the lungs using a systemic-to-pulmonary arterial shunt in infants with low birth weight may involve significant morbidity and mortality. We reviewed our experience with this procedure in patients weighing less than 3 kilograms.

Between June, 2002, and June, 2007, we placed systemic-to-pulmonary arterial shunts in 32 infants weighing less than 3 kilograms, the range being 1.8 to 2.86 kg, with a median of 2.5 kg. The median age at placement of the shunt was 8 days, with a range from 2 to 70 days. In 17 patients (53%), the anatomic defects had produced a functionally univentricular heart, while 15 (47%) had defects which permitted staging to biventricular repair. Patients staged to univentricular palliation were much more likely to have a circulation dependent on the arterial duct as compared with those staged to biventricular palliation (p < 0.001). The latter patients tended to have smaller pulmonary arteries, significantly the left pulmonary artery, which has a median diameter of 3.6 versus 2.0 mm, p = 0.01. In all patients a saphenous venous homograft was used as the conduit, its size ranging in diameter from 2.5 to 4 mm, with a median of 3.0 mm.

The overall hospital mortality rate for the entire cohort was 6.25%, with 2 patients dying. There was no significant difference between the two groups with regard to length of stay in intensive care or hospital. Follow-up has ranged from 3 months to 4.7 years, with a mean of 2.1 years). Of those with functionally univentricular hearts, 3 have subsequently died, along with 1 patient having a biventricular circulation (p = 0.3). All deaths occurred before takedown of the shunt. A trend toward longer survival was noted in those with biventricular as compared to functionally univentricular circulations (p = 0.06).

Systemic-to-pulmonary arterial shunts can be constructed safely in infants with biventricular physiology born with low weight. Those having functionally univentricular circulations carry an increased rate of mortality for the period of shunting. Using the saphenous venous homograft permits use of smaller grafts, which does not significantly increase the risk for thrombosis or survival when compared to previous studies using polytetrafluoroethylene grafts.