Syncope occurs frequently in adolescents, and is often benign. Potential worrisome syncopal events include those occurring with exertion, concurrent chest pain, dyspnoea or palpitations, and those with focal or diffuse neurologic changes.

A 16-year-old female was referred to our institution for a history of exercise-induced spells. She was diagnosed since the age of 2 years with neurocardiogenic syncope and postural orthostatic tachycardia syndrome. She had been evaluated at multiple institutions, and was followed by pediatric neurology for a diagnosis of migraines. Owing to recurrent worsening symptoms and a syncopal episode requiring resuscitation, an echocardiogram was performed. The right coronary was normal, but the left coronary artery ostium could not be identified well. Doppler patterns were suspicious of an abnormal left coronary artery, and computed tomography angiography was performed. This revealed a single coronary artery arising from the right aortic sinus, with the left coronary artery arising from the proximal coronary trunk and coursing through the infundibular septum. This was surgically treated utilising a left internal mammary artery bypass graft to the left anterior descending coronary artery. A year later, she has not experienced any recurrence of syncope, and has returned to athletic activity.

This case highlights the index of suspicion that must be present when evaluating any patient with syncope, both clinically and via echocardiography. A computed tomography angiogram is indicated for better evaluation of coronary artery anatomy when an anomalous coronary cannot be ruled out by echocardiography.

Successful management of bleeding disorders after congenital heart surgery requires detection of specific coagulation disturbances. Whole-blood rotation thromboelastometry (RoTEM®) provides continuous qualitative haemostatic profiles, and the technique has shown promising results in adult cardiac surgery.

To compare the performance of RoTEM® with that of conventional coagulation tests in children, we conducted a descriptive study in children undergoing congenital cardiac surgery. For that purpose, 60 children were enrolled and had blood samples taken before, immediately after, and 1 day after surgery. Conventional coagulation tests included: activated partial thromboplastin time, prothrombin time, fibrinogen, fibrin D-dimer, thrombin clotting time, factor XIII, and platelet count.

Post-surgical haemostatic impairment was present to some degree in all children, as seen by pronounced changes in activated partial thromboplastin time, prothrombin time, thrombin clotting time, and platelet count, as well as RoTEM® analysis. RoTEM® showed marked changes in clotting time – prolonged by 7–18% – clot formation time – prolonged by 46–71% – maximum clot firmness – reduced by 10–19%, and maximum velocity – reduced by 29–39%. Comparison of the two techniques showed that conventional coagulation tests and RoTEM® performed equally well with regard to negative predictive values for excessive post-operative drain production – more than 20 millilitres per kilogram per 24 hours after surgery – with an area under the curve of approximately 0.65.

RoTEM® can detect haemostatic impairments in children undergoing cardiac surgery and the method should be considered as a supplement in the perioperative care of the children where targeted transfusion therapy is necessary to avoid volume overload.

Congenital heart defect patients may experience neurodevelopmental impairment. We investigated their educational attainments from basic schooling to higher education.

Using administrative databases, we identified all Danish patients with a cardiac defect diagnosis born from 1 January, 1977 to 1 January, 1991 and alive at age 13 years. As a comparison cohort, we randomly sampled 10 persons per patient. We obtained information on educational attainment from Denmark's Database for Labour Market Research. The study population was followed until achievement of educational levels, death, emigration, or 1 January, 2006. We estimated the hazard ratio of attaining given educational levels, conditional on completing preceding levels, using discrete-time Cox regression and adjusting for socio-economic factors. Analyses were repeated for a sub-cohort of patients and controls born at term and without extracardiac defects or chromosomal anomalies.

We identified 2986 patients. Their probability of completing compulsory basic schooling was approximately 10% lower than that of control individuals (adjusted hazard ratio = 0.79, ranged from 0.75 to 0.82 0.79; 95% confidence interval: 0.75–0.82). Their subsequent probability of completing secondary school was lower than that of the controls, both for all patients (adjusted hazard ratio = 0.74; 95% confidence interval: 0.69–0.80) and for the sub-cohort (adjusted hazard ratio = 0.80; 95% confidence interval: 0.73–0.86). The probability of attaining a higher degree, conditional on completion of youth education, was affected both for all patients (adjusted hazard ratio = 0.88; 95% confidence interval: 0.76–1.01) and for the sub-cohort (adjusted hazard ratio = 0.92; 95% confidence interval: 0.79–1.07).

The probability of educational attainment was reduced among long-term congenital heart defect survivors.

This study aimed to describe the pre-operative and operative findings, as well as the post-operative haemodynamics of patients operated on for hypoplastic left heart. The findings of patients who survived or did not survive were also compared to anticipate the risk factors for mortality.

We retrospectively reviewed the anaesthetic and intensive care records of 11 (seven male and four female) patients who underwent a modified Norwood operation as neonates. There were eight patients who survived, while three did not survive after the operation. Haemodynamics, oxygenation, and medications of patients were recorded for the pre-operative and post-operative conditions for 2 days, and compared between groups of patients who survived and those who did not.

The normalised modified Blalock–Taussig shunt area was 3.28, 0.57 square millimetres per kilogram (mean, standard deviation) for the group of patients who survived and 3.55, 1.4 square millimetres per kilogram for the group of patients who did not survive (p = 0.51). The group of patients who survived had a significantly larger normalised aortic annulus area (3.3, 0.89 square millimetres per kilogram versus 1.68, 0.21 square millimetres per kilogram, p = 0.01), lower median age (5.57 (3–8) days versus 46.67 (4–90) days, p = 0.02), and lower weight (2.95, 0.46 kilograms versus 3.85, 0.56 kilograms, p = 0.03) than the group of patients who did not survive. Furthermore, the group of patients who did not survive had a significantly worse pre-operative condition, lower systemic venous and arterial oxygen saturation, and need for a high dose of drugs in the pre-operative and post-operative periods (p was less than 0.05 for each variable).

The pre-operative, operative, and post-operative findings may be related to mortality early after the modified Norwood operation for hypoplastic left heart syndrome.

The aim of this study is to evaluate our clinical experience using an Amplatzer septal occluder for catheter closure of a secundum atrial septal defect under transthoracic echocardiography guidance without general anaesthesia.

Patients eligible for transcatheter atrial septal defect closure were selected using transthoracic echocardiography. The largest defect diameter measured in different views was selected as the reference diameter. All procedures were performed under conscious sedation with fluoroscopic and transthoracic echocardiographic guidance.

Between November, 2006 and December, 2009 a secundum-type atrial septal defect was closed with the Amplatzer septal occluder in 40 patients with transthoracic echocardiographic guidance. The mean age and weight were 7.9 years and 26.9 kilograms, respectively. The mean atrial septal defect diameter was 11.4 millimetres, total septal diameter was 38.5 millimetres, and the mean device diameter and the difference between device and atrial septal defect diameter were 12.6 and 1.2 millimetres, respectively. There were no major complications. The mean follow-up time was 14.8 months.

In selected cases, in which the defects are small and the rims are adequate and transthoracic echocardiography provides high image quality, transthoracic echocardiography can be substituted with transoesophageal echocardiography. The ratio of defect size to total septal diameter can be used as a guide for patient selection; those that have a value of 0.33 or greater can be considered eligible for closure with transthoracic echocardiography. However, transthoracic echocardiography should not be used when there are large or multiple defects, or the rims are thin and soft and the image resolution is inadequate.

Since the introduction of percutaneous closure in the United States, rates of secundum atrial septal defect and patent foramen ovale closures have increased substantially. Whether or not closure rates are uniform or vary due to differences in regional practice patterns is unknown. We sought to investigate this by comparing regional rates of closure across Florida.

We identified all atrial septal defect closures from 2001 to 2006 in the Florida State Inpatient Database. Using small area analysis, zip codes were assigned to Hospital Referral Regions based on where patients were most likely to go for closure. We obtained population-normalised rates of overall, percutaneous, and surgical closure.

Of 1830 atrial septal defect and patent foramen ovale closures from 2001 to 2006, 751 were surgical and 1004 were percutaneous. The statewide closure rate was 1.91 per 100,000 people per year; regional rates varied 3.8-fold from 0.78 to 2.94 per 100,000 people per year. Percutaneous rates varied sevenfold from 0.25 to 1.75 per 100,000 people per year, while surgical rates varied 2.71-fold from 0.53 to 1.44 per 100,000 people per year.

Despite a consistent prevalence of atrial septal defects, and patent foramens ovale, rates of repair vary across regions, suggesting that closure is driven by provider practice patterns rather than patient pathology. Efforts should be directed towards increasing consensus regarding the appropriate, evidence-based indications for closure so as to avoid the costs and potential negative sequelae of over- or undertreatment.

The objective of this study was to evaluate the early and late results of atrioventricular valve surgery after Fontan operation.

Atrioventricular valve regurgitation is a known perioperative risk factor for Fontan operation. There are limited data on the outcomes of late atrioventricular valve surgery following Fontan operation.

Patients who underwent atrioventricular valve surgery following Fontan procedure were identified from the Mayo Clinic Fontan database. Medical records were reviewed for pre-operative, operative, and post-operative clinical and haemodynamic data. All patients not known to be deceased were sent health status questionnaires.

A total of 61 patients (28 females) underwent atrioventricular valve surgery following Fontan procedure. The median age at atrioventricular valve surgery was 14 years. The median duration between Fontan and atrioventricular valve surgery was 4.7 years. Median follow-up was 9 years. There were a total of 32 (52%) deaths with 8 (13%) within 30 days of surgery. The 5-, 10-, and 15-year survival rates were 67%, 57%, and 45%, respectively. On follow-up, 44 of 61 (72%) had arrhythmias, 21 of 29 (72%) were symptomatic, and 12 of 61 (20%) developed protein-losing enteropathy. On multivariate analysis, reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.

Atrioventricular valve surgery after Fontan procedure is associated with substantial late morbidity and mortality. Atrioventricular valve surgery in this cohort of patients portends poor long-term outcome and is associated with a high incidence of protein-losing enteropathy. Reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.

Acute rheumatic fever and its chronic sequelae, rheumatic cardiac disease, and neuropsychiatric movement disorders, remain major public health problems in South Africa. Early identification and treatment of streptococcal pharyngitis in susceptible individuals would prevent rheumatic cardiac disease. The B-cell antigen D8/17 is a marker of susceptibility to rheumatic fever in some populations.

We assessed the significance of the D8/17 marker in a group of South Africans. Blood was collected from 107 individuals; 40 patients had previous confirmed rheumatic fever, 20 were first-degree relatives, and 47 were controls. The expression of D8/17 in each sample was analysed by flow cytometry. The mean proportion of B-cells that were D8/17 positive was 0.5% in the index cases, 0.47% in their relatives, and 0.27% in the controls. There was a significant difference between the index cases and the controls, p = 0.03, but the mean percentage positive in each group was very low.

Patients with a history of rheumatic fever had statistically increased expression of the D8/17 marker. However, the actual percentages in this observational study were markedly lower than in other populations, ranging from 0.14%–1.53% compared to 11.6%–39.3%. The D8/17 marker would be an impractical screening tool in the South African population.

The aim of this study was to investigate the usefulness of ambulatory blood pressure monitoring versus head-up tilt test in the evaluation of children with a history of syncope.

We considered 146 consecutive children with more than one episode of syncope. All patients had a normal electrocardiogram at rest and were otherwise considered to be healthy. Forty-six patients,19 male, with a mean age of 13.6 plus or minus 5.6 years, were studied with a head-up tilt test and 100 patients, 41 male with a mean age of 9.4 plus or minus 5.6 years, were studied with ambulatory blood pressure monitoring. Twelve patients underwent both procedures. Hypotension during ambulatory blood pressure monitoring was defined when mean blood pressure values were lower than the 50th centile and the head-up tilt test was positive when syncope occurred. All patients were followed for 10 plus or minus 2 months.

Ambulatory blood pressure monitoring showed postural hypotension in 91% children, while head-up tilt test was positive for 54%. In the group of children having both tests, two of them were negative for both, 10 of 12 children had a positive ambulatory blood pressure monitoring while only five of 10 children had a positive response to head-up tilt test.

When a child with a normal resting electrocardiogram is referred with a typical history of syncope, the use of ambulatory blood pressure monitoring as a non-invasive first step for diagnosis of postural hypotension may be more sensitive than the head-up tilt test. Behavioural adjustments resolved the continued syncope in most cases. If episodes persist then the head-up tilt test is indicated.