Aims: To evaluate the quality of cardiac and surgical care provided for children with congenital cardiac malformations in the eastern county of Hungary. Methods: We used the method of clinical audit based in selection of criterions, developing five such criterions concerning timely diagnosis, access to treatment, and outcome. To examine compliance with these criterions, we analysed retrospectively the routine data relating to children living in Hajdu-Bihar County. The period of observation lasted from January, 1994, until December, 2001, and was divided into two epochs in order to evaluate any changes over time. Results: In the first epoch, 28 infants, representing 0.1% of all newborns, died of congenital cardiac disease, with one of the malformations being recognised post mortem. In the second epoch, 21 infants died, representing 0.09% of newborns. In this group, each malformation was diagnosed before death. In each period, 6 infants died without having undergone cardiac surgery, and having no other non-cardiac disease. The overall postoperative mortality was 9.2% for the first period, and 4.6% for the second. The number of patients waiting for non-urgent repair had reduced significantly by the end of the second epoch. Conclusions: The results show that the timely diagnosis of congenital cardiac malformations is in line with accepted international standards. Conversely, the access to invasive treatment was limited over the period of observation, albeit that the waiting list was reduced significantly by the end of the second epoch. The postoperative mortality for those suffering congenital cardiac malformations is also comparable with international standards, except for mortality during infancy for treatment of complex anomalies. Our audit highlights the need for surgical repair of the more complex malformations during infancy.

It is uncommon for thrombus to form within the heart of neonates with congenital cardiac disease. We describe a newborn with coarctation of the aorta, in whom a left atrial thrombus was discovered on the second day of life, and was thought to have been present before birth.

Objective: Intraoperative ultrasound was introduced to evaluate the adequacy of repair after surgical repair of congenital cardiac malformations. Our purpose was to review the evolution of this technique at our centre. Methods: We evaluated all intraoperative ultrasound studies undertaken between 1997 and 2002, reviewing the data from 1997 through 2001 retrospectively, but undertaking a prospective audit of studies undertaken from 2002 onwards. In all, we carried out a total number of 639 intraoperative ultrasound studies, from a possible 2737 cardiac operations (23.3%), using the epicardal approach in 580 (90.7%), and transoesophageal ultrasound in the other 59 patients (9.3%). Median age was 0.6 years, with an interquartile range from 0.06 to 3.6 years. Results: The findings obtained using intraoperative ultrasound influenced the surgical management in 63 of the 639 patients (9.9%), suggesting the need for additional surgery in 26, adjustment of the band placed round the pulmonary trunk in 16, preoperative assessment of the cardiac malformation in 5, and confirming the need for prolonged support with cardiopulmonary bypass for impaired ventricular function in 16. There were 18 early reoperations, 5 of which may have been predicted by intraoperative ultrasound. Of the 183 studies reviewed prospectively in 2002, it was not possible to obtain the complete range of views in 8 (4.4%), while in 27 patients (14.7%), the postoperative findings using transthoracic interrogation differed from the findings obtained immediately following bypass. Conclusion: Intraoperative ultrasound is an important technique for monitoring the results of complex congenital cardiac surgery. The immediate recognition of significant lesions, together with multidisciplinary discussion, allows for improved management and prevention of early surgical reintervention.

Objective: Orthotopic heart transplantation is considered a rescue option for children with failing staged palliation or repair of hypoplastic left heart syndrome. We present our strategy for management, and outcomes, for these complex patients. Methods: We transplanted 68 consecutive children, with diagnoses of hypoplastic left heart syndrome in 31, cardiomyopathy in 20, and post-operative complex congenital heart disease in 17. Of these patients, 9 (13.2%) were neonates, and 46 (67.6%) were infants. Median age was 118.5 days. Operative technique involves bicaval cannulation and anastamoses with continuous low flow bypass, and either short periods of circulatory arrest or continuous low flow antegrade cerebral perfusion for reconstruction of the aortic arch. Initial reperfusion of the donor heart utilizes glutamate and aspartate substrate enriched white blood cell filtered cardioplegia. Immunosuppressive therapy includes induction (pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin) and initial maintenance (calcineurin inhibitor, an anti-proliferative agent, and a weaning steroid protocol). Of the 31 patients with hypoplastic left heart syndrome, 23 underwent primary transplantation, and 8 underwent rescue transplantation from failing staged palliation in seven, or attempted biventricular repair in one. Of the seven patients who had failing staged palliation, three had undergone only the Norwood Stage 1 operation, 2 had undergone a Norwood Stage 1 operation and a Glenn superior cavopulmonary anastomosis and two had undergone a Norwood Stage 1 operation, a Glenn superior cavopulmonary anastomosis, and a completion Fontan operation. Results: The group undergoing primary transplantation was younger (p equals 0.007), weighed less (p equals 0.003), and waited longer for an appropriate donor heart (p equals 0.021) compared to those requiring rescue transplantation. No significant difference exists between the groups with regards to donor heart ischaemic time or post-transplant length of hospital stay. Thirty day survival (p equals 0.156) and overall survival (p equals 0.053) was better in those having primary transplantation, although these differences were not statistically significant when a p value of less than 0.05 is considered to be significant. In those having primary transplantation, no patients had elevated panel reactive antibody greater than 10%. Half of the 8 requiring rescue transplantation had panel reactive antibody greater than 10%, and this subgroup did especially poorly. Conclusion: Cardiac transplantation can offer children with failing staged palliation their only chance of survival. Transplantation, however, carries a high risk in this subgroup, especially in the setting of elevated panel reactive antibody.

We investigated the prevalence and forms of congenital cardiac malformations in six children with Kabuki syndrome. There were three girls and three boys, diagnosed at a median age of 1.7 years, with a range from 0.7 to 11.1 years. Cardiac lesions were present in five children (83%), specifically complete transposition, tetralogy of Fallot, coarctation of the aorta, ventricular septal defect, and patency of the arterial duct. Characteristic dysmorphic findings were noted in all patients, as well as a strong predisposition to severe problems with feeding in the neonatal period, and developmental delay. Cardiologists should be alert to this syndrome in children who present with the aforementioned constellation of findings, as patients with mild expression of Kabuki syndrome may go unrecognized for a considerable time.

In contrast to older patients, children and young adults rarely have isolated disease of the systemic atrioventricular valve. Stenosis and/or regurgitation of the systemic atrioventricular valve, however, frequently coexist with complex congenital cardiac disease. In addition, most patients undergoing surgery on the systemic atrioventricular valve have had previous intracardiac repairs.

Objective: To assess the impact of a computerised system to support decision-making concerning the management of warfarin used in maintenance of anti-coagulation. Design: Retrospective case series study comparing manual and computerised records of prescribing. Setting: A tertiary paediatric cardiology department in a teaching hospital. Participants: The 26 children receiving warfarin to maintain anticoagulation at the time of introduction of a computerised system to support decision-making. Interventions: A rules-based computerised system to support decisions, based on existing departmental guidelines, for management of anticoagulation using warfarin was introduced to aid prescribing physicians. Main outcomes: We assessed the stability of the International Normalised Ratio, along with the number of checks made of the ratio, and the adjustments of dosage. Dosages, and recheck interval prescriptions, were compared to the guidelines established by our department. Results: We compared 274 prescriptions made manually, and 608 made using the computerised system to support decision-making, covering periods of 4, and 11, months respectively. The mean proportion of time spent by the patients within their target range for the International ratio was maintained during the period studied, at 76 percent versus 79 percent (p = 0.79). The median number of checks of the ratio made for each patient over a period of 28 days was unchanged, at 1.9 versus 2.1 (p = 0.58). There was a significant change in prescribing practices, which more closely followed the departmental guidelines. Conclusion: The introduction of a computerised system to support decision-making maintained the stability of the International ratio using warfarin, without increasing the number of checks or adjustments of dosages, in a point-of-care service for anticoagulation in children.

It has long been known that the most complex combinations of cardiac malformations are those found in the setting of the so-called “splenic syndromes”.1 Many aspects of these syndromes have been controversial over recent years, not least the presence or absence of features of isomerism within the heart.2,3 Recent experience with genetic manipulation of mice, nonetheless, has now shown that it is possible to generate unequivocal evidence of cardiac isomerism, particularly in those animals which show features of right isomerism when the genes responsible for morphologically leftness are knocked out.4 Furthermore, when the crucial philosophical principle known as the “morphological method”5 is applied to the hearts of patients known to have visceral heterotaxy, it is equally clear that patients falling within these groups, when judged on the extent of the pectinate muscles relative to the atrioventricular junctions, exhibit isomerism of either the morphologically right or left atrial appendages.3

Most children with functionally univentricular hearts nowadays are treated surgically by creating a total cavopulmonary connection. In the resulting Fontan circulation, the venous return and the pulmonary arterial bed are coupled in series, bypassing the heart. This gives the potential for interaction between the abnormal circulation and function of the lungs. In this study, we investigated the pattern of impairment of pulmonary function, and its relation to decreased exercise capacity.

We performed spirometry in 33 (85 percent) of 39 eligible Norwegian children, aged from 8 to 16, with a total cavopulmonary connection, along with whole body plethysmography, the carbon monoxide single breath test, and a peak treadmill exercise test. The single breath test showed a mean corrected diffusing capacity of 66.5 percent of predicted, giving a z score of minus 2.88. The mean residual volume measured by whole body plethysmography was 146.8 percent, equivalent to a z score of 2.46, whereas the mean residual volume measured by the single breath test was 102.4 percent of predicted, this being the same as a z score of 0.43. The mean peak treadmill exercise test was 70.0 percent of predicted, equivalent with a z score of minus 3.07. Mean forced vital capacity was 85.7 percent of predicted, the equivalent z score being minus 0.92. Lung function correlated with the peak treadmill exercise test.

We have shown, therefore, that children with the Fontan circulation have reduced diffusing capacity, possibly caused by the abnormal circulation through the lungs. The difference between residual volume measured by plethysmography and the single breath test implies trapping of air. The correlation of parameters for lung function with peak consumption of oxygen during exercise indicates that the abnormalities of pulmonary function may affect physical capacity.

Various physiologic mechanisms have been proposed to account for the development of hypoplasia of the left heart. The mechanism thus far most widely accepted suggests that the entity starts as severe or critical aortic stenosis during fetal gestation. Obstruction at the level of the abnormal aortic valve is then held to increase left ventricular afterload, resulting in decreased systolic and diastolic function. Shunting across the patent oval foramen is then reversed, so that blood flows from left to right. This reversal of flow during fetal gestation decreases the volume of blood crossing the mitral valve, thus decreasing the further potential for growth of the left ventricle.1 Additional support for this postulated physiologic mechanism was provided with the advent of fetal echocardiography during the 1980s.2–4 It was the group of Allan, working at Guy's Hospital in London, which first documented the fetal development of hypoplasia of the left heart by serial echocardiographic observation.4 In their retrospective study of 7000 pregnancies, 462 fetuses were diagnosed to have a structural cardiac defect at the time of the initial echocardiogram. Among those, 28 patients had dilated and dysfunctional left ventricles and aortic valves. The majority of these patients were also found to have concomitant endocardial fibroelastosis. Out of 15 patients in the series who were followed with serial echocardiograms, five progressed to develop hypoplasia of the left heart. With echocardiographic technology undergoing refinement over the same period, it was during this era that the first fetal cardiac intervention was performed using echocardiographic guidance.2,5,6 With still further technologic advances, fetal diagnosis of hypoplasia of the left heart can now be made as early as 13 weeks gestational age.7 One entity which is frequently associated with the hypoplastic left ventricle and aortic stenosis is endocardial fibroelastosis. There is an overlap of pathology between these three entities.8–10 In this report, we describe our own experience in intervention in a fetus suspected of developing hypoplasia of the left heart.

Tissue Doppler measurements of the right and left lateral ventricular walls were made before and after perforation of the pulmonary valve using radiofrequency energy in a patient with pulmonary atresia and intact ventricular septum. The ratio of peak tissue velocity during rapid ventricular filling to atrial contraction increased for both atrioventricular valves after perforation of the pulmonary valve, and the patient was able to be weaned off prostaglandins without further intervention. Such measurements made using tissue Doppler may aid in the management of patients with pulmonary atresia and intact ventricular septum by predicting improvements in right ventricular relaxation.

The study was designed to investigate the value of assessing right ventricular volume by transoesophageal 3-dimensional echocardiographic techniques compared with the standard transoesophageal cross-sectional approach. Echocardiography was performed using a multiplane probe. The 3-dimensional data sets were reconstructed after electrocardiographic and respiratory gated scanning, calculating the 3-dimensional volumes by the method of multiple slices. Cross-sectional determination of volume was performed using a modified area-length method, and the biplane multiple slice method following Simpson's rule. We studied 15 patients, with ages ranging from 6 to19 years, and body surface areas from 1.1 to 1.67 square metres. It proved possible top determine volumes with both methods in all patients. As determined by 3-dimensional echo, volumes were greater, being 113.0 plus or minus 61.2 millilitres at end-systole, and 61.7 plus or minus 36 millilitres at end-diastole, than those calculated from cross-sectional images using Simpson's rule, which gave values of 92.5 plus or minus 52 millilitres, and 41.3 plus or minus 22 millilitres. Compared to the values obtained using the area-length method, at 116.9 plus or minus 61 millilitres, and 60.3 plus or minus 30 millilitres, there were only small differences at end-systole, with a bias of 1.4, and limits of agreement of 20.9 millilitres, as well as at end-diastole, when bias was minus 3.8, and limits of agreement 22.3 millilitres. Correlation was also good, with coefficients of 0.93, and 0.91, respectively. The mean difference between the volumes by 3-dimensional acquisition and the multiple slice method was larger, with higher limits of agreement, at end-diastole showing bias of 20.5, and limits of agreement of 30.1 millilitres, and for end-systole bias of 20.4, and limits of agreement of 32.2 millilitres. Our data confirm that cross-sectional echocardiographic assessment of right ventricular volumes in children with atrial septal defects is quick, and reasonably reliable in clinical practice when employing the area-length method.

Real-time three dimensional echocardiography is increasingly used for imaging patients with congenital cardiac malformations. One of the limitations of a transthoracic approach is that intervening structures can impact on the quality of the images obtained. We hypothesised that, during surgery, epicardial three-dimensional echocardiography would provide images of high quality. We report our findings in eight children or young adults, with weights ranging from 2.8 to 70 kilograms, in whom we used this approach. In all cases, we obtained images of good quality, which could be analysed rapidly in the operating room. Of the eight cases, seven had echocardiographic findings which matched exactly the surgical findings. The remaining child had been diagnosed echocardiographically with a cleft in the aortic leaflet of the mitral valve, but was found at surgery to have a double orifice in the valve.

It is now well recognized that patients fulfilling the diagnostic criterions for the group of hearts usually described as atrioventricular canal malformations, or atrioventricular septal defects, can present with shunting at atrial level, at both atrial and ventricular levels, and on occasion, with shunting only at ventricular level.1,2 It is also well recognized that, in most instances, the patients with shunting exclusively at atrial level have separate atrioventricular valvar orifices for the right and left ventricles, this arrangement often described as the “ostium primum” variant of atrial septal defect.3 Morphological and echocardiographic studies, however, have shown that, in this variant presumed to represent deficient atrial septation, it is the atrioventricular septal structures, rather than the atrial septum, which are deficient, the phenotypic feature being the presence of a common atrioventricular junction.4,5 In this review, we will show how, using modern day echocardiographic techniques, particularly the newly developed potential for three-dimensional display, it is an easy matter to identify the presence or absence of the common atrioventricular junction, and then to demonstrate the various relationships between the valvar leaflets, the septal structures, and the common junction itself which determine the options for clinical presentation within the group.

Background: We describe alternative surgical techniques for construction of systemic-to-pulmonary arterial shunts, and propose criterions for their application in selected patients. Patients and methods: We constructed a variety of modified systemic-to-pulmonary arterial shunts, using polytetrafluroethylene grafts, in 92 selected patients with cyanotic congenital heart disease and anomalies of the aortic arch and systemic veins. Their age ranged from 7 days to 3.6 years, with a mean of 7.08 months. We performed 88 operations through a thoracotomy. Of this cohort, 60 patients underwent a second-stage operation, with 15 receiving a superior cavopulmonary connection, 16 a total cavopulmonary connection, and 29 proceeding to biventricular repair after a mean interval of 15.6 months. We have 21 patients awaiting their second or final stage of palliation. Results: There were five early (5.4%) and six late deaths (6.8%), two of which were related to construction of the shunts. At a mean follow-up of 45.29 months, the increase in diameter of pulmonary trunk and its right and left branches was uniform and significant (p value less than 0.001). Pulmonary arterial distortion requiring correction at the time of second-stage operation was observed in 5 patients (6.1%). Adequate overall palliation was achieved in 98% of the cohort at 8 months, 91% at 12 months, and 58% at 18 months. Conclusions: Patients with a right- or left-sided aortic arch and right-sided descending thoracic aorta, those with anomalies of systemic venous drainage masking the origin of great arterial branches, and those withdisproportionately small subclavian arteries, constitute the ideal candidates for our suggested modification of the construction of a modified Blalock-Taussig shunt. The palliation provided by these shunts was satisfactory, with predictable growth of pulmonary arteries, insignificant distortion in the great majority, and easy take-down.

We evaluated the efficiency of non-invasive magnetic resonance coronary angiography in detecting coronary arterial lesions in 106 patients, aged from 4 months to 37 years, with a median of 13 years, with Kawasaki disease. Non-contrast enhanced, free-breathing magnetic resonance coronary angiographic studies using both the steady-state free precession technique, namely bright blood imaging, and navigator-echo proton density weighted black blood imaging, so-called black blood imaging, were performed in all the patients. Conventional X-ray coronary angiography was performed in 70 patients with coronary arterial lesions.

We observed 97 aneurysms, 17 dilatations, 17 occlusions, 18 localized stenoses and 10 recanalized vessels, and we clarified their unique pattern of images on magnetic resonance coronary angiography. The differences in size of the aneurysms as seen on X-ray coronary angiography and bright blood imaging was mean 0.0, and the 95% confidence interval was from −1.4 to 1.5 on the Bland-Altman plots. With bright blood imaging, the sensitivity of occlusion and localized stenosis based on X-ray angiography was 94.2% and 97.2%, specificity was 99.5% and 97.2%, and negative-predictive value was 99.5% and 97.2%, respectively. Black blood imaging provided remarkable visualization of the thickened intima of aneurysms, and/or thrombus, in 38 lesions. We conclude that magnetic resonance coronary angiography can visualize all types of lesions due to Kawasaki disease in patients of all ages, and that it is useful to reduce the number of times X-ray angiography needs to be performed in patients with Kawasaki disease.

A newborn girl with atresia of the common pulmonary vein, presented immediately after birth with severe cyanosis and acidosis. The diagnosis of totally obstructed total pulmonary venous return was made by cross-sectional echocardiography. Subsequent cardiac catheterization failed to demonstrate the site of pulmonary venous return. Necropsy showed the pulmonary veins to be connected bilaterally to an atretic common pulmonary vein. There was no obvious alternative pathway for pulmonary venous return.

Introduction: Recurrent but infrequent syncopes represent a diagnostic challenge, since they frequently remain unexplained despite extensive investigations. This applies specifically for patients who carry an increased risk of potentially lifethreatening arrhythmias, either due to congenital cardiac disease or primary electrical disorders. Implantable loop recorders permit long-term electrocardiographic monitoring. Experience with these devices is still limited in children. Patients and methods: Between January 1999 and August 2005, 12 patients underwent implantation of a loop recorder in our tertiary referral centre. The mean age was 10.9 years, with a range from 2 to 17 years. Of the patients, 6 had structural disease, 3 had primary electrical abnormalities, and 3 had no cardiovascular disease. Results: Resyncope occured in 9 of the 12 patients. Arrhythmic origin of the syncope was diagnosed in 4 of these patients. The events recorded were ventricular fibrillation in 2, intermittent asystole in 1, and pacemaker-syndrome in the other patient. Malignant arrhythmia was ruled out in the remaining 5 patients. There were no complications related to implantation of the loop recorder, and the mean duration until explantation was 8.3 months. Conclusions: Based on our experience, we suggest that implantation of a loop recorder represents an additional tool for a selected group of children. Due to its invasive nature, it should be restricted to patients at high risk, or those in which there is substantial clinical suspicion of the likelihood of serious arrhythmias when conventional testing has been inconclusive. In this cohort, implantation of the loop recorder either helps to establish the correct diagnosis, or to exclude an arrhythmic event, thus avoiding unnecessary escalation of therapy and providing reassurance for the family.

Background: Disturbances of conduction are well known in the setting of acute rheumatic fever. The aim of this study is to investigate the QT dispersion as seen in the surface electrocardiogram of children with acute rheumatic fever. Methods: QT dispersion was quantitatively evaluated in 88 children with acute rheumatic fever. Patients were divided into two groups based on the absence or presence of carditis. As a control group, we studied 36 healthy children free of any disease, and matched for age with both groups. Repeat echocardiographic examinations were routinely scheduled in all patients at 3 months after the initial attack to study the evolution of valvar lesions. Results: The mean QT dispersion was significantly higher in children with rheumatic carditis. But there was no statistical difference between children without carditis and normal children. Among the children with carditis, the mean dispersion was higher in those with significant valvar regurgitation. Dispersion of greater than 55 milliseconds had a sensitivity of 85%, and specificity of 70%, in predicting rheumatic carditis, while a value of 65 milliseconds or greater had sensitivity of 81% specificity of 85% in predicting severe valvar lesions in acute rheumatic carditis. At follow-up examination, a clear reduction on the QT dispersion was the main finding, reflecting an electrophysiological improvement. Conclusions: These observations suggest that QT dispersion is increased in association with cardiac involvement in children with acute rheumatic fever.