Oral sildenafil has been demonstrated to be an effective treatment for pulmonary hypertension, and is increasingly used in children. We report an infant with pulmonary hypertension, stable on regular treatment with oral sildenafil, who presented in acute respiratory failure after aspiration, requiring ventilation and intensive care. The course of the stay in intensive care was difficult, with recurrent pulmonary hypertensive crises despite use of oral sildenafil, use of 100% oxygen, high frequency oscillatory ventilation, and inhaled nitric oxide. In view of his instability, and the presumed inability to absorb the sildenafil orally due to gastrointestinal malabsorption, sildenafil was administered as a continuous intravenous infusion. With this therapy, it proved possible to wean from oxygen, nitric oxide, and ventilatory support. Intravenous sildenafil, therefore, might be an effective alternative for children with pulmonary hypertension during episodes of acute deterioration and malabsorption, preventing life-threatening pulmonary hypertensive crises. Its pharmacokinetics, efficacy, and safety, nonetheless, need to be validated in randomized controlled trials.

Chronic illness in a child produces stress for both the child with the illness and the family of which he or she is a part.1 Today, it is estimated that greater than one-tenth of children are living with some form of chronic illness or condition.2–3 Faced with this stress, children and families are required to adapt to potential physical, emotional, social, and financial challenges. Professionals providing health care have an opportunity to influence how children and families interpret and adapt to these challenges. Guidance can be drawn from the multiple theoretical perspectives that have explored the process of adaptation to chronic illness.

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.

Anomalous drainage of the right superior caval vein into the morphologically left atrium as an isolated cardiac malformation is a rare anomaly. Most patients present with cyanosis. Thus far, about 20 cases have been reported in the literature. We report a case of cyanosis due to this malformation in a male neonate, which was complicated by the meconium aspiration syndrome. The malformation was diagnosed by echocardiography and cardiac catheterization. Surgery resulted in complete recovery.

Iatrogenic thrombosis within the heart is rare following intracardiac operations. We undertook surgery to correct a 30-month-old girl with Fallot's tetralogy. After a period of 6 months following the operation, echocardiography revealed a thrombus measuring 16 by 12 millimetres attached to the tendinous cords supporting the antero-superior leaflet of the tricuspid valve. Medical treatment having failed, we proceeded to surgical excision so as to avoid pulmonary embolisation. Histological examination confirmed the thrombotic nature of the mass.

Three patients with heart failure after chronic right ventricular apical pacing were treated with resynchronization. Biventricular pacing was used for two patients, and the other was treated with left univentricular pacing. In all patients, we observed a dramatic improvement of left ventricular dimension, function, and clinical state. We conclude that biventricular or left ventricular pacing is superior to right ventricular apical pacing in children who are pacemaker-dependent.

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.

Surgical intervention for hearts with transposition, defined as concordant atrioventricular and discordant ventriculo-arterial connections, has been one of the landmark achievements in the field of paediatric cardiac surgery. In the early 1950s, pioneer surgeons attempted to palliate patients with transposed arterial trunks with an early form of the arterial switch operation. As a result of initially dismal outcomes secondary to difficulties with coronary arterial transfer, the unprepared nature of the morphologically left ventricle, and primitive methods for cardiopulmonary bypass, the arterial switch was abandoned in favour of several procedures achieving correction at atrial and venous levels, culminating in the Mustard and Senning operations.1,2 These innovative procedures produced the earliest surviving children with transposition. Although the atrial switch procedures achieved widespread acceptance and success during the mid-1960s through the mid-1980s, the search for an operation to return the great arteries to their normal anatomic positions continued. This pursuit was stimulated primarily by the accumulating observations in mid-to-late term follow up studies of: an increasing frequency of important arrhythmic complications, including sinus nodal dysfunction, atrial arrhythmias, and sudden, unexplained death, by the development of late right ventricular dysfunction and significant tricuspid regurgitation in a ventricle potentially unsuited for a lifetime of systemic function by a small but important prevalence of obstruction of the systemic and/or pulmonary venous pathways, and by dissatisfaction with the operative mortality in the subgroup of infants complicated by additional presence of a large ventricular septal defect.3–6 As we have already discussed, a number of novel procedures to achieve anatomic correction had been described as early as 1954, but clinical success was not accomplished until 1975, when Jatene and co-workers7 astounded the world of paediatric cardiology with their initial description.

Acute ingestion of large quantities of alcohol is known to be able to trigger acute myocardial infarction. A 19-year-old boy presented at the emergency department of our hospital with complaints of severe retrosternal chest pain. One night before this event, he had drunk large amounts of alcohol. The level of alcohol in his blood was measured at 0.59 grams per litre. A 12-lead electrocardiogram showed elevations of the ST segment, averaging from 2 to 10 millimetres, in leads V1-6, DI and aVL. Since consumption of alcohol is very common in the community, the triggering effect of binge-drinking and consumption of large amounts of alcohol on acute myocardial infarction should be considered as a crucial subject for public health so as to raise the consciousness of the population, especially young persons.

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.

A baby presented at term with respiratory distress was managed with extracorporeal membrane oxygenation. Bronchoscopy revealed tracheal hypoplasia, complete tracheal rings, and agenesis of the right main bronchus. Echocardiography showed a left pulmonary arterial sling arising from the proximal part of the right pulmonary artery. Cardiac catheterization demonstrated abnormal pulmonary vasculature in the left lung which would have prevented survival, even after surgical repair. Diagnostic catheterization was important in delineating the anatomy, and aided in the decision not to proceed with surgical repair.

For patients requiring intervention because of progressive disease of the aortic valve, the perfect palliation will provide a valve that produces normal dynamics of flow, will not require anti-coagulation, will grow with the patient, and have long term durability. Current surgical interventions include aortic valvoplasty, or replacement with either a mechanical or tissue prosthesis. Options for tissue valves include insertion of a pulmonary autograft in the Ross procedure, a cadaveric homograft, or porcine or bovine xenograft valves. The optimal option is still debated.