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.

At this time, the current practice for treatment of patients with isolated ventricular septal defect is infrequently studied. With this in mind, it was our intent to assess the current management of ventricular septal defect at a single center, The Children's Hospital, Denver. We reviewed the practice at this institution to determine if there is an evidence base for when or if a patient with an isolated ventricular septal defect requires surgical repair. With approval from the Colorado Multiple Institutional Review Board (protocol # 06-0097), we reviewed the data on patients with isolated ventricular septal defect seen during the calendar years of 2004 and 2005, determining the state of the patients, and the level of intervention through December 31, 2005.

We describe the findings in a six-year-old girl who presented with signs of left atrioventricular valvar insufficiency. The echocardiogram showed a common atrioventricular junction, intact atrial and ventricular septal structures. At surgery, the left-sided atrioventricular valve was found to be tri-foliate, and corrected by valvoplasty. To the best of our knowledge, this is the first case of atrioventricular septal defect with common atrioventricular junction and intact septal structures diagnosed during life.

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.

A long with prematurity and chronic lung disease, the presence of congenital cardiac disease in infants and young children is a significant risk for the clinical consequences of an illness produced by infection with the respiratory syncytial virus.1 In this review, we present a current understanding of such illnesses, their prevention, and their treatment.

Background: Inappropriate systemic inflammatory endothelial and leucocyte activation is a major pathogenic component of post-operative low cardiac output syndrome (LCOS) and associated pulmonary oedema, low urine output, and generalised oedema, in paediatric cardiac surgery. We hypothesised that acute pre-treatment with a parenteral nutrition component omega-3 fatty acid infusion in-vitro, would prevent the pattern of leucocyte and endothelial activation seen in paediatric cardiac surgery through induction of preconditioning. Methods: Endothelial cells were isolated from saphenous vein of 75 adult cardiac surgery patients, grown in monolayers, pre-treated, and then stimulated with either endotoxin (LPS), TNFalpha, or complement C5a (prime mediators of systemic inflammation with paediatric cardiac surgery). Endothelial production of the neutrophil recruiting cytokines IL-6, IL-8, and endothelial expression of the neutrophil adherence receptors E-Selectin, ICAM-1 were assessed. The effects on the inflammatory pro-coagulant response of the endothelium i.e. loss of surface thrombomodulin and production of tissue factor were also examined. Similar methods were employed using isolated peripheral blood neutrophils. The effects on endothelial nuclear translocation of the acute inflammatory transcription factor NFkB and the heat shock protein (HSP72) response were investigated for mechanistic insight. Results: Four hours pre-treatment with the omega-3 infusion prevented endothelial release of neutrophil recruiting cytokines IL-6 and IL-8, also prevented upregulation of endothelial receptors required for neutrophil adherence E-Selectin and ICAM-1, and maintained surface anticoagulant thrombomodulin levels, in response to stimulation with LPS (Table 1) or TNFalpha.

“All the vital mechanisms, however varied they may be, have only one object, that of preserving constant the conditions of life in the internal environment.”1

An essential function of the cardiopulmonary system is to generate sufficient flow of oxygenated blood around the circulation in order to maintain normal cellular metabolism. The systemic delivery of oxygen is a function of the cardiac output and the content of oxygen in the systemic arterial blood, while the extent to which metabolising tissues require this oxygen for the maintenance of their integrity and function defines the systemic consumption of oxygen. As metabolising tissues have no mechanism for storing oxygen, they depend on its continuous supply, which must at least match their changing demands. As a result, it is a fundamental requirement of survival that the systemic consumption of oxygen, at all times, is matched by appropriate levels of its delivery.

As patients with congenital cardiac malformations increasingly survive therapeutic interventions, and our understanding of primary electrical diseases increases, the landscape of paediatric and congenital electrophysiology is expanding. Electrophysiologic abnormalities, both tachycardic and bradycardic, are commonly seen in post-operative patients with congenital cardiac disease, as well as being part of the natural history of congenital malformations and cardiomyopathies. Disturbances of rhythm represent an increasing morbidity in this population, and therapies using devices in the form of pacemakers and implantable cardioverter-defibrillators have taken on a correspondingly important role. In this review, we discuss some of the key features and recent advances in pacing for bradycardia, resynchronization pacing, anti-tachycardia pacing, and use of implantable cardioverter-defibrillators.