The activation of inflammatory systems as a consequence of cardiopulmonary bypass represents one possible means by which inj ury to tissues and associated dysfunction of organs may occur in the postoperative cardiac patient, both pediatric and adult. Kirklin and colleagues demonstrated in the early 1980's that complement activation occurs as a consequence of blood exposure to the extracorporeal circuit. Subsequent studies demonstrated this to be true in systems using both bubble and membrane oxygenators. The anaphylatoxin C5a and its metabolite C5a des arg are produced during activation of the complement cascade. They are potent stimulants which may induce activation of neutrophils. This activation may be manifested as changes in shape, decreased cellular deformability, and changes in adhesive and secretory function. Activation of neutrophils by these fragments of complement could contribute to neutrophilmediated damage to tissues by promoting retention of neutrophils in vascular beds and secretion of cytotoxic substances. Recent studies demonstrating the release of neutrophil granular enzymes into the plasma following cardiopulmonary bypass are evidence that activation of neutrophils is taking place in this setting.

To assess the clinical impact of percutaneously implanted balloon expandable endovascular stents on patient management, we reviewed 20 children aged 14 days to 4.8 years (median 1.3 years) with residual vascular obstructions in the immediate postoperative period. Patients included 11 with pulmonary arterial stenosis, five with stenosis of venous pathways after a modified Fontan procedure or bidirectional cavopulmonary anastomosis, and four with a restrictive modified Blalock-Taussig shunt. Placement of the stent was optimal in 18 of 20 patients (24 of 26 Palmaz or Palmaz-Schatz implants). In two patients, rupture of the balloon resulted in malposition of the stent. Reoperation was avoided and symptomatic improvement was noted in 11 of 20 patients (55%), while procedural complications occurred in eight patients. Seven of 10 patients presenting with a low cardiac output syndrome died despite relief of the obstructions. This early experience supports consideration of the application of these devices in the management of significant vascular obstructive lesions in the immediate postoperative period, thus avoiding early reoperation in this profoundly compromised population.

Background: Elevation of cardiac troponin I in the serum is a specific marker for myocardial injury. We measured levels of troponin I in the serum in children before and after cardiac catheterization to determine if this procedure was associated with an increase in levels of troponin. Methods: We enrolled patients under 21 years of age undergoing cardiac catheterization at our institution. A baseline sample of serum was drawn at the start of the procedure. Repeat samples were obtained immediately after, and six hours subsequent to the procedure. All samples were analyzed for cardiac troponin I using the Abbott AxSYM microparticle immunoassay system. Levels were considered normal (0–0.4 ng/ml) or elevated (>0.4ng/ml). Patients were excluded if the baseline level was elevated. Results: Levels of cardiac troponin I were elevated in the serum from 11 of 14 (79%) cases immediately after the procedure (p < 0.0001), and in 12 of 14 (86%) six hours later (p < 0.0001). Only 2 patients had recognized complications potentially causing myocardial injury. Conclusion: Levels of cardiac troponin I increase in the serum in a high proportion of children after cardiac catheterization. These elevations can be observed immediately, and are maintained for at least six hours. Our study suggests that cardiac catheterization, predominantly intervention, is associated with myocardial injury, even in the absence of complications.

The size of the coronary arteries parallels the ventricular mass, thus it may be abnormal in complete transposition beyond infancy and could be influenced by the arterial switch operation. To investigate this possibility, we measured the diameters of the right, left main trunk, anterior descending, and circumflex coronary arteries before and three to seven years (mean 4.8) after the arterial switch operation in 17 patients with a “normal” distribution of the coronary arteries (so-called Shaher type 1). The values were compared with 18 controls who had Kawasaki disease with no apparent coronary arterial disease. The right, left anterior descending, and circumflex arteries were smaller than control values before the operation. The post-/preoperative ratios of the diameter were 1.16±0.11 for the right coronary artery, 1.18±0.16 for the left main trunk, 1.20±0.18 for the left anterior descending artery, and 1.22±0.26 for the circumflex artery. There were no significant differences among these values. After surgery, the right coronary artery was larger, but the left coronary arteries were smaller in the patients than in the controls: 2.5±0.3 vs 2.0±0.2 mm for the right coronary artery; 2.4±0.3 vs 2.7±0.1 mm for the left main trunk; 1.9±0.2 vs 2.4±0.2 mm for the left anterior descending artery; 1.6±0.4 vs 2.2±0.5 mm for the circumflex artery, respectively. The posterior descending coronary artery originated from the right coronary artery in all patients. The total cross-sectional area of the right coronary, left anterior descending, and circumflex arteries was 9.7±2.4 mm2 in the patients, and 11.8±2.9 mm2 in the controls (p>O. 1), suggesting that the increased size of the right coronary artery compensates for the small left coronary arteries. We conclude that the arterial system in complete transposition, with a large right coronary artery and small left coronary system, remains smaller than normal even at midterm follow-up after anatomic repair despite normalization of left ventricular volume and muscle mass.

A retrospective analysis of 55 patients who underwent replacement of the left atrioventricular valve following surgery for atrioventricular septal defect with separate right and left atrioventricular valves or with a common valvar orifice has revealed a marked decrease in the need for valvar replacement over time. Between 1983 and 1987, the incidence of replacement following repair has been 3% in infants. Overall early mortality for the entire series, extending from 1970 to 1987, was 22%. Mortality in the first year of life was 58%, which was significantly greater than mortality beyond one year of age (14%, p<0.01). Obstruction of the left ventricular outflow tract was a frequent incremental risk factor for death during infancy. The incidence of complete heart block (25%) is disappointingly high, and may reflect distortion of the left atrioventricular junction in an attempt to place as large a prosthesis as possible. The incidence of complete heart block has not decreased with time. These results confirm the desirability of secondary valvoplasty procedures to enable a child to be larger before ultimate replacement of the valve is required.

Distinguishing the benign and malignant cardiac masses is usually a diagnostic challenge, and safe, non-invasive, and reliable techniques are necessary to avoid any delay in treatment. The previously described “sun ray” appearance is an important sign in the diagnosis of cardiac angiosarcoma, and in this report we present 3-T magnetic resonance imaging findings to distinguish the rare but fatal cardiac angiosarcoma.

We describe a case of influenza A myocarditis and transient left ventricular wall thickening in a 14-year-old girl presenting with acute heart failure. Admission echocardiogram revealed significant left ventricular hypertrophy with depressed left ventricular systolic function. The aetiology of the ventricular thickening was demonstrated to be myocardial oedema using cardiac magnetic resonance imaging. The natural course of this unusual clinical presentation of acute myocarditis and the importance of cardiac magnetic resonance imaging in this challenging clinical setting are discussed.

A 21-week gestational age foetus was diagnosed with left ventricular non-compaction, Ebstein’s anomaly, sinus bradycardia, first-degree heart block, and agenesis of the ductus venosus. The prognosis was guarded given the constellation of findings, and the foetus was monitored closely. Despite a potentially poor outcome, the foetus survived. Prognosis in foetally diagnosed left ventricular non-compaction is usually poor; however, rarely, foetuses can survive postnatally.

Cardiac rhythm monitoring has been facilitated by the use of implantable loop recorders. New models of these devices are 87% smaller than before allowing for easier implantation and use in the paediatric population. Recommendations are for closure with adhesive. We report a device extrusion in a 6-year-old patient. Based on this, our practice has changed to include subcutaneous sutures this complication.

Sudden cardiac death is the most common mode of death during childhood and adolescence in hypertrophic cardiomyopathy, and identifying those individuals at highest risk is a major aspect of clinical care. The mainstay of preventative therapy is the implantable cardioverter-defibrillator, which has been shown to be effective at terminating malignant ventricular arrhythmias in children with hypertrophic cardiomyopathy but can be associated with substantial morbidity. Accurate identification of those children at highest risk who would benefit most from implantable cardioverter-defibrillator implantation while minimising the risk of complications is, therefore, essential. This position statement, on behalf of the Association for European Paediatric and Congenital Cardiology (AEPC), reviews the currently available data on established and proposed risk factors for sudden cardiac death in childhood-onset hypertrophic cardiomyopathy and current approaches for risk stratification in this population. It also provides guidance on identification of individuals at risk of sudden cardiac death and optimal management of implantable cardioverter-defibrillators in children and adolescents with hypertrophic cardiomyopathy.

We hypothesised that lower mesenteric near-infrared spectroscopy values would be associated with a greater incidence of gastrointestinal complications in children weighing <10 kg who were recovering from cardiac surgery. We evaluated mesenteric near-infrared spectroscopy, central venous oxygen saturation, and arterial blood gases for 48 hours post-operatively. Enteral feeding intake, gastrointestinal complications, and markers of organ dysfunction were monitored for 7 days. A total of 50 children, with median age of 16.7 (3.2–31.6) weeks, were studied. On admission, the average mesenteric near-infrared spectroscopy value was 71±18%, and the systemic oxygen saturation was 93±7.5%. Lower admission mesenteric near-infrared spectroscopy correlated with longer time to establish enteral feeds (r=−0.58, p<0.01) and shorter duration of feeds at 7 days (r=0.48, p<0.01). Children with gastrointestinal complications had significantly lower admission mesenteric near-infrared spectroscopy (58±18% versus 73±17%, p=0.01) and higher mesenteric arteriovenous difference of oxygen at admission [39 (23–47) % versus 19 (4–27) %, p=0.02]. Based on multiple logistic regression, admission mesenteric near-infrared spectroscopy was independently associated with gastrointestinal complications (Odds ratio, 0.95; 95% confidence interval, 0.93–0.97; p=0.03). Admission mesenteric near-infrared spectroscopy showed an area under the receiver operating characteristic curve of 0.76 to identify children who developed gastrointestinal complications, with a suggested cut-off value of 72% (78% sensitivity, 68% specificity). In this pilot study, we conclude that admission mesenteric near-infrared spectroscopy is associated with gastrointestinal complications and enteral feeding tolerance in children after cardiac surgery.

The justification for the introduction of the arterial switch procedure was based, primarily, on concern regarding the long-term ability of the right ventricle to perform as the systemic pumping chamber. In this article, the functional performance of both the systemic and pulmonary ventricles after atrial redirection procedures and the arterial switch operation will be discussed.