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.

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.

Aims: Bosentan is efficacious in idiopathic pulmonary arterial hypertension, and the variants associated with connective tissue disease, but not currently approved for treatment of pulmonary arterial hypertension due to Eisenmenger's syndrome. We sought to evaluate its effect in adults with Eisenmenger's syndrome. Methods: We administered bosentan on the basis of compassionate use in 23 patients with Eisenmenger's syndrome, aged 37 plus or minus 14 years. Of the patients, 17 had never received specific treatment for pulmonary arterial hypertension, five were transitioned from treprostinil, and one from beraprost to bosentan. We measured functional class, saturation of oxygen, haemoglobin levels and six-minute walk distance at baseline, one, six months and at most recent follow-up. Results: Baseline functional class was IV in three, III in fifteen, and II in five patients. At follow-up, with a mean of 15 plus or minus 10 months, 13 of the 23 patients (57%) had improved by at least one functional class, from a median baseline of III to II (p equal to 0.016), mean saturation of oxygen at rest had increased from 81% to 84% (p equal to 0.001), and levels of haemoglobin had decreased from 178 plus or minus 26 grams per litre to 167 plus or minus 19 grams per litre (p equal to 0.001). Overall, the six-minute walk distance did not change from baseline of 335 metres. The distance walked by those not previously receiving specific therapy, however, improved from 318 plus or minus 129 to 345 plus or minus 123 metres (p equal to 0.03). Conclusion: Treatment of adults with Eisenmenger's syndrome using bosentan significantly improved functional class, saturation of oxygen at rest, and decreased levels of haemoglobin. Treatment with bosentan was associated with improvement in six-minute walk distance in those not previously receiving specific therapy. In patients already in receipt of specific therapy, transition to bosentan resulted in no clinical deterioration.

Background: To determine the clinical relevance of change in the level of lactate in the serum as a meanings of establishing the efficacy of the circulation immediately after the Fontan procedure in small children. Methods: We measured the concentration of lactate in the serum consecutively in 30 patients undergoing the Fontan procedure, without fenestration, under the age of 2 years. Of these, we did not use cardiopulmonary bypass in 13. Results: Concentrations gradually increased during the first several hours after establishment of the Fontan circulation, and then eventually decreased, reaching the normal range within 48 hours, with specific values of 0.9 plus or minus 0.3 pre-operatively, 2.3 plus or minus 1.1 immediately after creation of the Fontan circulation, 4.0 plus or minus 2.4 at 6 hours, 1.6 plus or minus 0.6 at 24 hours, and finally 1.3 plus or minus 0.4 millimoles per litre at 48 hours. This trend was irrespective of use or no use of cardiopulmonary bypass. The higher initial level in the intensive care unit was related to higher systemic venous pressure, higher transpulmonary pressure gradient, higher maximal level of alanine transaminase and blood urea nitrogen, and longer duration of peritoneal drainage, the latter as a monitor of sequestration of fluid rather than peritoneal dialysis. In patients with initial levels greater than 3.0 millimoles per litre, there was extended duration of endotracheal intubation. Conclusions: Postoperative changes in the levels of lactate in the serum subsequent to creation of the Fontan procedure were peculiar, not necessarily correlated with the cardiac output, but relevant to the clinical course.

Aims: Our purpose was to assess the value of magnetic resonance angiography as a non-invasive alternative to catheterization in the evaluation of patients with tetralogy of Fallot, including those with pulmonary atresia. Methods and results: We evaluated prospectively, using magnetic resonance angiography, 30 patients, aged from 1 to 18 years, 15 with tetralogy of Fallot and pulmonary stenosis, and 15 with pulmonary atresia. The studies obtained using magnetic resonance provided adequate visualization of the aorta, and provided excellent imaging of the pulmonary trunk and its right and left branches. Compared with catheterization, magnetic resonance had 100 percent sensitivity, specificity and accuracy for defining the presence or absence of the pulmonary arteries. Magnetic resonance also had 93.9 percent sensitivity, 98.2 percent specificity, and 96.7 percent accuracy for detection of stenosis or hypoplasia of the pulmonary arteries. We detected 25 major aortopulmonary collateral arteries with magnetic resonance, but only 22 with conventional angiography. There was complete agreement between the two methods in detecting patency of the arterial duct in 6 patients, and of Blalock-Taussig shunts in 12 patients. Conclusion: Magnetic resonance angiography is a useful tool in the evaluation of patients with tetralogy of Fallot. It can be considered a non-invasive alternative to cardiac catheterization in the evaluation of the pulmonary vascular anatomy.

Surgical waiting lists are of high importance in countries, where the national health system is unable to deliver surgical services at a rate that would allow patients to avoid unnecessary periods of waiting. Prioritization of these lists, however, is frequently arbitrary and inconsistent.

The objective of our research was to analyze the medical decision-making process when prioritizing patients with congenital cardiac malformations for cardiac surgical procedures, identifying an appropriate representation of knowledge, and transferring this knowledge onto the design and implementation of an expert system (“PrioHeart”).

The medical decision-making process was stratified into three stages. The first was to analyze the details of the procedure and patient to define important impact factors on clinical priority, such as the risk of adverse events. The second step was to evaluate these impact factors to define an appropriate “timing category” within which a procedure should be performed. The third, and final, step was to re-evaluate the characteristics of individual patients to differentiate between those in the same timing category.

We implemented this decision-making process using a rule-based production system with support for fuzzy sets, using the FuzzyCLIPS inference engine and expert system shell as a suitable development environment for the knowledge base.

The “PrioHeart” expert system was developed to give paediatric cardiologists a tool to allow and facilitate the prioritization of patients on the cardiosurgical waiting list. Evaluation of “PrioHeart” on limited sets of patients documented appropriate results of prioritization, with a significant correlation between the prioritization made using “PrioHeart” and those results obtained by the individual consultant specialist.

We conclude that our study has demonstrated the feasibility of using an expert system approach with a fuzzy, rule-based production system to implement the prioritization of cardiac surgical patients. The approach may potentially be transferable to other medical subspecialities.

We report a case in which a meandering right pulmonary vein connecting to the left atrium is associated with hypoplasia of the right lung, horseshoe lung, abnormal pulmonary lobation, and abnormal branching of the pulmonary arteries. We discuss its relationship to the so-called scimitar variant, and to the scimitar syndrome itself.

We report an unusual case in which the peripheral part of the otherwise normal left lung was supplied by major systemic arteries arising from the left subclavian, internal mammary and coeliac arteries. Unlike most cases with systemic arterial supply to a normal or sequestered lung, where the systemic arterial branches get access to the lung through the pulmonary hilum or inferior pulmonary ligament, in our patient the systemic arterial branches entered the lung through the transpleural routes.

We report a rare variant of the Scimitar syndrome, in which the right lower pulmonary vein takes a meandering course before finally connecting in normal fashion to the left atrium. The pulmonary parenchymal segment drained by the tortuous vein is supplied by a systemic collateral artery, which was coiled via a catheter. We also closed the intracardiac defects by surgery. The elder brother of the patient had classical Scimitar syndrome.

Background: Babies with cardiovascular malformations are usually asymptomatic at birth. Earlier diagnosis is likely to lead to a better outcome. This study examines trends in the timing of diagnosis of potentially life-threatening cardiovascular malformations. Methods: Ascertainment of all cardiovascular malformations diagnosed in infancy in the resident population of one English health region in 1985–2004. Babies considered to have a life-threatening cardiovascular malformation included all those with hypoplastic left heart, pulmonary atresia with intact ventricular septum, transposition of the great arteries, or interruption of the aorta; and babies dying or undergoing operation in the first 28 days with coarctation of the aorta, aortic stenosis, pulmonary stenosis, tetralogy of Fallot, pulmonary atresia with ventricular septal defect, or total anomalous pulmonary venous connection. Results: Of 688,167 live born babies in the 20 years of the study, 4444 had cardiovascular malformations diagnosed in infancy (6.5 per 1000). Cardiovascular malformations were potentially life-threatening in 685 (15%). Over the time of the study, 8% were recognised prenatally, 58% postnatally before discharge from hospital, 31% in life after discharge and 3% after death. Malformations most likely to remain undiagnosed at discharge were coarctation of the aorta (63%), interruption of the aortic arch (50%), and total anomalous pulmonary venous connection (47%). Over the 20 years of the study, the proportion of babies diagnosed antenatally increased from around 1% to 20% and no case was first diagnosed after death in the last 10 years. However, the recent proportion going home without a diagnosis remains around 25%. Conclusions: Overall one third of babies with potentially life-threatening cardiovascular malformations leave hospital undiagnosed. In recent years better antenatal diagnosis has reduced this proportion to one quarter. Better early recognition of such babies is unlikely to be achieved by clinical examination and is more likely to come from further improvements in antenatal diagnosis and more widespread adoption of routine pulse oximetry.

Introduction: Repair of Tetralogy of Fallot up until recent decades involved aggressive resection and annular enlargement through a right ventriculotomy. This resulted in ventricular scarring and pulmonary incompetence, with an increased risk of ventricular tachyarrhythmia and sudden death in young adulthood. Following the NICE guidelines, implantation of ICDs as primary prevention in patients with repaired Tetralogy is ever increasing. This study aims to determine the rate of appropriate and inappropriate discharges, the success rate of ICD therapy and the impact of ICD implantation on the use of anti-arrhythmic medication in this population of patients. Materials and Methods: This is a retrospective review of patients with repaired Tetralogy of Fallot (n = 18) and pulmonary stenosis (n = 2) with implantable cardioverter defibrillators managed at our tertiary centre. Patients were identified from our outpatient database, their notes and charts were examined and details regarding indication for ICD implantation, device specifications and complications following implantation were collected. Data was also collected on the incidence of appropriate and inappropriate therapies and the success rate of ICD therapy along with the impact of implantation on the usage of anti-arrhythmic medication in these patients. Results: Of the 20 patients, 18 had previous repair of Tetralogy of Fallot and 2 had pulmonary valvotomy and infundibular resection for pulmonary stenosis between 1969 and 1989. 70% (n = 14) of these patients required reoperation with 10 patients having pulmonary valve replacements (PVR), 3 having redo infundibular resections and 1 requiring aortic valve replacement. At the time of consideration for ICD implantation 80% had moderate to severe pulmonary incompetence and 60% had more than mild right ventricular dilatation on echocardiography. Indications for ICD implantation were symptomatic ventricular tachycardia requiring cardioversion (n = 8), ventricular tachycardia on 24 hr tape/Reveal or electrophysiological study (n = 8), ventricular fibrillation (VF)/pulseless ventricular tachycardia (VT) (n = 2) and syncope with an abnormal EPS other than VT (n = 2, high grade ventricular ectopics, sinus node dysfunction).

The median age at implantation was 22 years (16.4–43 years). All our patients had dual chamber devices implanted with either dual (n = 13) or single coil (n = 6) ventricular leads. GEM3 AT (n = 5), Marquis DR (n = 8) and Maximo DR (n = 7) generators (Medtronic Inc.) were implanted in sub pectoral position and both anti-tachycardia pacing and cardioversion modes were programmed as part of individualised VT and VF protocols. Early post procedural complications included atrial lead displacement (n = 1) and pneumothorax requiring drainage (n = 1).

During a median follow up of 1.6 years (0.03– 4.5 years) several episodes of inappropriate therapies were noted in 6 patients (30%) especially early after implantation. This was found to be mainly due to atrial tachyarrhythmia, double counting of T waves or inaccurate interpretation of varying PR intervals as AV dyssynchrony which were effectively dealt with by changes in device programming. There were 33 episodes of inappropriate anti-tachycardia pacing (ATP) in 4 patients and 19 episodes of inappropriate cardioversion in 5 patients. Appropriate ATP was instituted in 4 patients (25%) with successful termination of all 20 episodes (100% success rate) of ventricular tachycardia. One patient required cardioversion with successful termination of VF. One patient (5%) with troublesome tachyarrhythmia died suddenly of unknown cause, 10 months after AICD implantation having had no detections or therapies on his device.

Prior to ICD implantation 8 patients were on amiodarone therapy. At the time of last follow up after AICD implantation all patients were established on anti-arrhythmic agents and of these 6 patients were on amiodarone with the others being effectively managed on beta-blockers and/or flecainide.

Late complications of ICD implantation included lead failure in 1 patient requiring replacement 3.3 years after implantation and generator replacement in a patient who was pacemaker dependent a year after implantation due to an advisory issued by the manufacturer regarding the risk of sudden battery depletion. Conclusions: In our study we found a rate of 0.6 appropriate and 1.4 inappropriate therapies (0.9 episodes of inappropriate ATP and 0.5 episodes of inappropriate cardioversion) per patient-year of follow up following ICD implantation which is in keeping with published literature. The mortality in our study group was 5% which is acceptable given the high risk population. Implantation of an ICD allowed switching over from amiodarone to less toxic anti arrhythmic therapy in a proportion of patients. Anti-tachycardia pacing was very successful in terminating tachyarrhythmia in our population with 100% success in terminating ventricular tachycardia.

Objective: In recent years blood transfusion has become a debated health care issue. To minimise exposure to infectious agents and reduce bank blood transfusion requirements, leucocyte filtration and perioperative red cell salvage (RCS) are increasingly used in paediatric patients. We hypothesised RCS would reduce the need for additional blood products in children following cardiopulmonary bypass (CPB). Methods: Patients undergoing routine or emergency cardiac surgery requiring CPB over a study period of 3 months were included prospectively in the analysis. Haemoglobin, platelet count, coagulation screen and heparin levels were performed before, immediately after surgery and 24 hours later. RCS was performed in theatre according to surgical and anaesthetic preference. Red cells were salvaged from the surgical site, anticoagulated, washed and following resuspension in saline reinfused into the patient within 4 hours. The incidence of post-operative bleeding (>10 ml/kg/hr) was recorded, as was the need for additional red blood cells, platelets and fresh frozen plasma (FFP). The need for blood products was at the discretion of the consultant intensivist. Statistical analysis was performed using student t-test and Chi squared methods. Significance was accepted as p < 0.05. Results: Thirty-five consecutive patients (34.54 ± 43.55 months, 13.48 ± 14.39 kg) were included in the analysis. A total of 17 infants <12 months were included, 9/24 who received RCS and 8/11 who did not (p 0.052). Cyanotic heart disease was seen in 40%. RCS was performed in 24 of 35 patients, who were significantly older (44.2 ± 44.1 vs. 13.6 ± 25.5, p 0.02) and heavier (16.6 ± 16.2 vs. 6.7 ± 4.7, p 0.01). No difference was seen in the prevalence of cyanosis between the two groups. Post-operative bleeding was seen in 21% who underwent RCS and 40% in those who did not (p 0.33). The need for additional red blood cells was significantly reduced in those who received RCS, 37.5% vs. 91%, p 0.003, as was the use of FFP, 8.3% vs. 45.5%, p 0.02. There was no difference in the need for platelet transfusion, p 0.2. Discussion: In this study RCS was performed on 68.5% of children following CPB. RCS significantly reduced the need for further blood and FFP transfusion, although this was not related to post-operative bleeding. This has important implications for both exposure to infectious agents and health economics. That children who underwent RCS were older and heavier may be related to the complexity of surgery and CPB in younger patients, although infants were represented in both groups. A further analysis of potential health and economic benefits in a homogenous group is needed.

Aim: To present the experience of the UK Pulmonary Hypertensive Service for Children at Great Ormond Street Hospital in treating children with “Eisenmenger” type physiology with the dual endothelin receptor antagonist bosentan. Patients and methods: Fourteen inoperable patients were included in the study. These were selected based on a history of an unrestrictive intracardiac communication and established right to left shunt at rest. There was a female: male ratio 1: 0.75 and age range at initiation of treatment of 7–18.6 years (mean 12.5 yrs). Six patients had Trisomy 21. The cardiac diagnoses were: 6 large VSDs, 6 CAVSD (1 of which was not Trisomy 21) and 2 more complex congenital heart disease cases that underwent palliative surgery (DILV, DOLV, TGA, PA banding and DORV, TGA, VSD, PS, Waterston). Twelve patients received antiplatelet/anticoagulation therapy. WHO functional class, haemoglobin (Hb), oxygen saturation, 6 min walk tests, echocardiograms and ECGs were assessed before starting treatment with bosentan and after 6 and 12 months of follow up. Twelve patients had a cardiac catheterisation before treatment commenced. The parameters were compared for patients before treatment and at 6 and 12 months post treatment with paired t-test and ANOVA. Results: All patients were severely symptomatic the mean WHO functional class being 3.2 (range 3–4). The mean Hb was 18.3 g/dl (range 13.4–22.9). The mean PVR of all patients was 30.98 units m2 (7.6–57). The Trisomy 21 patients had a mean PVR of 34.4 units m2 and non Trisomy 21 patients had a mean of 28.6 units m2. Mean oxygen saturation at rest was 78.9% (range 67–93), mean 6 min walk test distance was 223.8 m (n = 10, range 108–360) and the lowest oxygen saturation during the walk test was 59.7% (42–79) at 6 min or below. Mean duration of therapy was 15.2 months (range 6–34 months). Bosentan was well tolerated in all patients but discontinued in one due to non compliance with monthly screening blood tests. All patients remained stable and their echocardiogram and ECG findings were unchanged throughout. After 6 months mean WHO functional class had decreased significantly to 2.96 (p = 0.029), whilst Hb and oxygen saturation at rest had not changed. Ten of the 14 patients were on treatment for more than a year and after 12 months the WHO functional class was maintained at the same improved level. The Hb and oxygen saturation at rest remained unchanged. After 12 months the 6 min walk mean distance had increased from 211 to 253 metres (non significant). Conclusion: Patients treated with bosentan for up to 1 year showed clinical improvement and remained stable. After 6 months treatment WHO functional class had significantly improved and improvement was sustained for up to 1 year.

Background: Paediatric heart failure can be difficult to assess. Symptoms vary widely in children who have poor ventricular function on echocardiogram. In addition exercise testing is not possible in young children. BNP has been used in adult heart failure and more recently in paediatrics. We investigated BNP in the setting of a paediatric heart failure and transplantation service to assess its usefulness as a clinical marker of heart failure. Method: Clinical and echocardiographic data were correlated to 126 BNP samples. Patients were 3 weeks to 16 years of age. Left ventricular end-diastolic dimension (LVEDd) was related to normal values for body surface area (z-score). Clinical status was defined using Ross and NYHA scores. Thirty four samples were from patients with normal ventricular function. Of the remainder, most were from patients with idiopathic dilated cardiomyopathy (38), anthracycline cardiotoxicity (15), congenital heart disease (25), viral myocarditis (6) and restrictive cardiomyopathy (6). Analysis: BNP was correlated to parametric data (fractional shortening (FS%)) with pearsons correlation coefficient. For non-parametric data (z-score, NYHA and Ross score), spearmans correlation coefficient was used. Results: Rising BNP levels correlate to deteriorating clinical status with significance to the 0.01 level (Ross and NYHA), Figure 1. There was also correlation, significant to the 0.01 level to FS%, Figure 2. Increasing BNP levels correlated to the increasing LVEDd z-score significant to the 0.05 level. Mean BNP: in ventricular dysfunction 634 pg/ml (S.E.M. = 80), in normal function 11.9 pg/ml (S.E.M. = 1.6). Conclusions: This is the largest study of BNP in paediatric heart failure. The stronger correlation of BNP to NYHA and ROSS than to LVEDD and FS% suggests a useful role in assessment of children with heart failure. We believe it is useful in the outpatient setting particularly when care is shared with general paediatricians. It also appears to be a useful addition in the assessment of heart failure and perhaps timing of transplantation in a specialist centre.