Background: Little is known regarding parental preference for a one-stage complete repair versus a two-staged approach with initial palliation, followed by repair, of the congenital cardiac malformation. Methods: We interviewed 103 parents of healthy children referred to a clinic for pediatric cardiology. Participants were presented with a hypothetical scenario in which their children had a cardiac lesion requiring surgery. The surgery could be performed either by means of one-stage complete repair, or using a two-stage approach, with palliation first followed by complete repair a year later. The mortality rate for the one-stage repair was set at 5%. Participants were asked to choose between the one- and two-stage approaches, with differing mortality rates for the two-stage approach. The scenarios included options when the two-stage combined mortality rate was lower than the one-stage mortality, and the first stage mortality rate was at 1% and 3%, and when the two-stage combined mortality rate was the same as that for one-stage mortality, these being set at 1% and 3%. Results: When the two-stage combined mortality rate was lower than that of the one-stage repair, participants were more likely to choose the two-stage approach if the first stage mortality rate was 1% as compared to 3% (57% and 44%, respectively, p = 0.04). When the two-stage combined mortality rate was the same as the one-stage approach, participants choosing the two-stage approach when the mortality rate was set at 1%, and when it was raised to 3%, were not significantly different (42% and 34%, respectively, p = 0.24). When the combined two-stage mortality was the same as that set for one-stage repair, participants with no insurance were less likely to choose the two-stage approach than those covered by insurance (p = 0.03). Conclusions: In the chosen scenarios, when the mortality for a two-stage combined approach is the same as that for one-staged repair, more parents choose the one-staged repair. If the two-stage combined mortality is lower than that for one-staged repair, parents are more likely to choose the two-stage repair if the mortality for the first stage is lower. When the mortality rates for the one-stage and two-stage approaches are the same, people without insurance are more likely to choose one-staged repair.

Background and purpose: There is little documented evidence of young people with congenital cardiac disease being consulted as to what help, if any, they really need in relation to their condition. Most research concentrates on the medical aspects of the condition. There are studies of psychological and social functioning, but few have directly sought the opinions of the young people. More recent research has indicated a need for health professionals to develop services to meet both psychosocial and physical needs of young people with congenital cardiac disease. The findings of the recent Kennedy Report support this need. The purpose of this study was to explore the views of young people with a range of congenital cardiac conditions, on what would help them better deal with their condition, and when and how help might be provided. Methods: This was a qualitative study using semi-structured interviews. We interviewed 16 young people in their own home. Interviews were tape-recorded and transcribed. Analysis was conducted using the “Framework” method. Results: Whilst most of the young people interviewed actively manage their condition, they think more support from others would be beneficial. Issues of activity and communication were cited most often as areas requiring more understanding from people they interact with, for example teachers and peers. The discussion focuses on how health professionals might change or develop their practice to help young people better cope with their condition. Conclusions: Most of the young people interviewed in this study had developed their own strategies for coping with their condition. Although this is a small study, the young people provided important suggestions as to how health professionals could better develop current services.

Background and purpose: Although much previous research has focused on the medical aspects of congenital cardiac disease, there is a growing body of research which suggests that families may need help and support with the wider issues associated with the medical condition. We have previously ascertained from young people with congenital cardiac disease their views on this subject. The purpose of this study was to obtain the views of their parents about the need for, and shape of, services for rehabilitation. Methods: This was a qualitative study using semi-structured interviews. We interviewed 17 parents in their own home. Interviews were tape-recorded and transcribed. Results: Parents would welcome more help and support from health professionals to enable them to manage more effectively the condition with their children. Particular areas of concern relate to the information they receive about the condition; communication between themselves and health professionals; establishing safe levels of activity; and managing the condition at school. Conclusions: This is a small study of the experiences and views of parents, which provides some important information on ways in which health professionals could address the current lack of services for rehabilitation.

We designed a multi-hospital prospective study of children less than 12 years to determine the comparative clinical profile, severity of carditis, and outcome on follow up of patients suffering an initial and recurrent episodes of acute rheumatic fever. The study extended over a period of 3 years, with diagnosis based on the Jones criteria. We included 161 children in the study, 57 having only one episode and 104 with recurrent episodes. Those seen in the first episode were differentiated from those with recurrent episodes on the basis of the history. The severity of carditis was graded by clinical and echocardiographic means. In those suffering their first episode, carditis was significantly less frequent (61.4%) compared to those having recurrent episodes (96.2%). Arthritis was more marked in the first episode (61.4%) compared to recurrent episodes (36.5%). Chorea was also significantly higher in the first episode (15.8%) compared to recurrent episodes (3.8%). Sub-cutaneous nodules were more-or-less the same in those suffering the first (7%) as opposed to recurrent episodes (5.8%), but Erythema marginatum was more marked during the first episode (3.5%), being rare in recurrent episodes at 0.9%. Fever was recorded in approximately the same numbers in first (45.6%) and recurrent episodes (48.1%). Arthralgia, in contrast, was less frequent in first (21.1%) compared to recurrent episodes (32.7%). A history of sore throat was significantly increased amongst those suffering the first episode (54.4%) compared to recurrent episodes (21.2%). When we compared the severity of carditis in the first versus recurrent episodes, at the start of study mild carditis was found in 29.8% versus 10.6%, moderate carditis in 26.3% versus 53.8%, and severe carditis in 5.3% versus 31.8% of cases, respectively. At the end of study, 30.3% of patients suffering their first episode were completely cured of carditis, and all others showed significant improvement compared to those with recurrent episodes, where only 6.8% were cured, little improvement or deterioration being noted in the remainder of the patients. We conclude that the clinical profile of acute rheumatic fever, especially that of carditis, is milder in those suffering their first attack compared to those with recurrent episodes.

Background: Cardiac remodelling is now recognised as an important aspect of cardiovascular disease progression and is, therefore, emerging as a therapeutic target in cardiac failure due to different etiologies. Little is known about the influence of different therapies for cardiac failure on the remodelling seen in infants with congenital cardiac disease. Methods: During follow-up of a prospective and randomized trial, we investigated therapeutic effects on neurohormonal activation, ventricular function, and myocardial gene expression. We compared the data from 8 infants with severe congestive heart failure due to left-to-right shunts, who received digoxin and diuretics alone, to 9 infants who received additional treatment with propranolol. Results: In these infants, β-adrenergic blockade significantly reduced highly elevated levels of renin, from 284 ± 319 μU/ml compared to 1061 ± 769 μU/ml. Systolic ventricular function was normal in both groups, but diastolic ventricular function was improved in those receiving propranolol, indicated by significantly lower left atrial pressures, lower end-diastolic pressures, and less pronounced ventricular hypertrophy, the latter estimated by lower ratios of myocardial wall to ventricular cavity areas on average of 42%. Further hemodynamic parameters showed no significant differences between the groups, except for the lower heart rate in infants treated with propranolol. In those treated with digoxin and diuretics, there was a significant downregulation of β2-receptor and angiotensin-2 receptor genes, and up-regulation of endothelin A receptor and connective tissue growth factor genes, that were partially prevented by additional treatment with propranolol. Conclusions: β-blockade is a new therapeutic approach for congestive heart failure in infants with congenital cardiac disease, producing with significant benefits on neurohormonal activation, diastolic ventricular function, and cardiac remodelling.

Background: Minimally invasive cardiac surgery is now becoming standard in the correction of simple congenital cardiac malformations. We introduced a clinical pathway for fast track recovery of school activities in children after minimally invasive cardiac surgery, and assessed the function of the pathway in children with atrial or ventricular septal defects, comparing minimally invasive surgery to repair through a conventional full sternotomy. Methods: We studied 15 children of school age who underwent repair of an atrial or ventricular septal defect through a lower midline sternotomy, and 10 children undergoing repair through a full sternotomy. The clinical pathway was for extubation to take place in the operating room, echocardiographic evaluation on the 5th postoperative day, and discharge home on the 7th postoperative day, with return to school within 2 weeks, and resumption of all gymnastic activity within 6 weeks of the minimally invasive surgery. Results: In those having a lower midline sternotomy, postoperative hospital stay was 7.4 ± 0.8 days, with return to school 8.0 ± 2.4 days after discharge. They resumed gymnastics 41 ± 11 days after the minimally invasive surgery. In those having a full sternotomy, in contrast, these parameters were 13.5 ± 2.7, 23.1 ± 8.4, and 95 ± 43 days, respectively. Of the 15 children undergoing a minimally invasive approach, 12 (80%) fulfilled the criterions of our clinical pathway. Conclusions: We conclude that minimally invasive cardiac surgery can safely be performed in children. In addition to its cosmetic role, the technique has added value in promoting early return to normal school life, including gymnastics.

Objective: To evaluate the feasibility of the arterial switch for surgical repair of transposition, defined as the combination of concordant atrioventricular and discordant ventriculo-arterial connections, after late referral. Methods: From March 2000 to August 2001, six children underwent an arterial switch procedure following left ventricular preparation because of late referral. The mean age at referral was 8.3 months, with a range from 3 to 25 months, and mean body weight was 5.3 kg, with a range from 3.7 to 9.3 kg. The mean saturation of oxygen was 57%, with a range from 50 to 72%. Associated defects included a restrictive ventricular septal defect in three patients, aortic coarctation in one, and partially anomalous pulmonary venous connection in one. The mean interval between referral and the arterial switch procedure was 3.7 months, within a range from 1 to 7 months. A mean of 1.5 surgical procedures were undertaken to prepare the left ventricle, the most being 3 procedures, including combinations of creation of an inter-atrial communication in four patients, banding of the pulmonary trunk in five, and creation of a systemic-to-pulmonary arterial shunt in three. We evaluated left ventricle ejection and shortening fractions, left ventricular diastolic diameter and volume, right and left ventricular wall thicknesses, and the ratio of right to left ventricular values by echocardiography at referral, immediately before, and one week after the arterial switch procedure. Results: All children are alive and well, with a mean follow-up of 17 months, ranging from 9 to 26 months. Echocardiography showed a statistically significant decrease of the ratio between right and left ventricular wall thicknesses, from 1.33 ± 0.26 at referral to 0.79 ± 0.08 before the switch procedure (p < 0.005). Left ventricular function was adequate after arterial switch, with a mean ejection fraction of 79.3%, ranging from 66 to 87%, and a mean shortening fraction of 41.7%, ranging from 30 to 49%. Conclusions: Despite late referral, and initially inadequate left ventricular volume and mural thickness, children with transposition can successfully be treated with the arterial switch procedure, provided that the left ventricle is adequately prepared, using echocardiography to monitor left ventricular morphology and function.

Background: We have used intravascular ultrasound in an attempt to clarify the extent of vascular remodelling of the aortic arch after the repair of aortic coarctation by use of a subclavian flap. Methods: We investigated 13 patients with coarctation of the aorta, ranging in age from 1.4 to 43.0 months, with a mean of 20.8 months, who underwent aortoplasty by incorporation of a subclavian flap. The mean postoperative period was 19.6 months, with a range from 0.03 to 41.2 months. The luminal morphology of the aortic arch was evaluated by intravascular ultrasound at the time of post-operative catheterization. Results: We observed 3 cases longitudinally. Over the period of observation, we found three types of morphology of the aorta at the site of incorporation of the subclavian flap, namely a snowman shape with two inflection points, a pisiform shape with one inflection point, and a round shape without any points of inflection. There was a correlation between the cross-sectional shapes at the site of the subclavian flap in the postoperative period (p < 0.01). In each case, we measured the cross-sectional area at the site of subclavian flap, at the descending aorta, and at the distal aortic arch. The cross-sectional area, and the increment of the cross-sectional area at the site of subclavian flap, was larger. Conclusion: The shape of the lumen subsequent to repair of aortic coarctation changes progressively from a snowman, to a pisiform, and finally to a round shape. Greater growth of the subclavian flap compared to the native wall of the aorta was observed for at least the first 4 years after repair. This finding may improve our understanding of the remodelling process of the arterial trunks after surgical repair.

Aims: Our aims were to use transthoracic three-dimensional echocardiography to assess the morphology of atrial septal defects in children prior to closure, and to compare the three-dimensional echocardiographic data with transcatheter and surgical findings. Methods and results:We used transthoracic three-dimensional echocardiography in 62 consecutive patients, aged from 2 to 18 years, with atrial septal defects, measuring the maximal diameter and the extent of the rims. Subsequent to the study, we referred 42 patients for transcatheter closure, the rims being measured at greater than 4 mm. We found a good correlation between the maximal diameter of the defect as measured at transthoracic three-dimensional echocardiography and using a balloon (y = 3.45 − 0.73x; r = 0.78; p < 0.0001), the mean difference between the measurements being 2.4 ± 2.8 mm. Successful closure with the Amplatzer septal occluder, having a mean size of 22 ± 4 mm, was achieved in 95% of the patients. Of the original cohort, 20 patients were referred for surgical closure. In these patients, the inferior rim had been deemed insufficient in 5, the postero-superior rim in 6, and the postero-inferior rim in 9. Complete agreement was found when the deficiency of the rim as judged using transthoracic three-dimensional echocardiography was compared with intraoperative findings. The correlation between measurements of the deficiency of the rim achieved by transthoracic three-dimensional echocardiography and at surgery was excellent (y = 0.2 + 0.98x; r = 0.93; p < 0.0001), the mean difference between the measurements being no more than 0.6 ± 0.4 mm. Conclusions: Transthoracic three-dimensional echocardiography proved accurate in measuring the maximal diameter and rims of atrial septal defects within the oval fossa. This non-invasive method will be valuable in selecting children for transcatheter or surgical closure of such defects.

During the last two decades, several advances have resulted in marked improvement in medium-term survival, with excellent quality of life, in children undergoing cardiac transplantation. Improved outcomes reflect better selection of donors and recipients, increased surgical experience in transplantation for complex congenital heart disease, development of effective surveillance for rejection, and wider choice of immunosuppressive medications. Despite all of these advances, recipients continue to suffer from the adverse effects of non-specific immunosupression, including infections, induction of lymphoproliferative disorders and other malignancies, renal dysfunction, and other important end-organ toxicities. Furthermore, newer immunosuppressive regimes, thus far, appear to have had relatively little impact on the incidence of chronic rejection. Progress in our understanding of the immunologic mechanisms of rejection and graft acceptance should lead to more targeted immunosuppressive therapy and avoidance of non-specific immunosupression. The ultimate goal is to induce a state of tolerance, wherein the recipient will accept the allograft indefinitely, without the need for long-term immunusupression, and yet remain immuno-competent to all non-donor antigens. This quest is currently being realized in many animal models of solid organ transplantation, and offers great hope for the future.

We report a rare variant of anomalous origin of the left coronary artery from the non-adjacent sinus of the pulmonary trunk. The patient also had the right coronary artery arising from the non-facing sinus of the aorta.

We report two siblings with isolated ectopic hearts. Neither child had associated congenital diseases. To the best of our knowledge, this is the first reported familial occurrence of ectopic hearts.

An 8-month-old boy presented with right hemiplegia of sudden onset after 20 days of Kawasaki disease, which was not initially treated by gamma globulin. Cranial X-ray computed tomography confirmed cerebral infarction as the cause of the right hemiplegia. In subsequent weeks, he developed multiple thromboses in coronary aneurysms. He successfully underwent intracoronary thrombolysis using tissue plasminogen activator without haemorrhagic complications. Cerebral infarction as a complication of Kawasaki disease is rare, and is a difficult clinical situation to manage.

We report an infant with a large perimembranous ventricular septal defect, who had two separate orifices in the tricuspid valve, each supported by separate cordal apparatus, detected incidentally during surgery.