Remote ischaemic pre-conditioning and cardiac ischaemic post-conditioning provide myocardial protection in cardiac surgery. However, these two endogenous strategies have not been directly compared in a clinical setting. The purpose of this study was to compare the efficacy of remote ischaemic pre-conditioning and post-conditioning in providing myocardial protection to children undergoing cardiopulmonary bypass for surgical repair of ventricular septal defect.

We randomly assigned 60 paediatric patients scheduled for surgical correction of congenital ventricular septal defect to the post-conditioning group (n = 20), remote pre-conditioning group (n = 20), or control group (n = 20). Post-conditioning consisted of 30 seconds of ischaemia and 30 seconds of reperfusion achieved by clamping and unclamping the aorta, repeated three times over 3 minutes immediately after cardioplegic arrest. Remote ischaemic pre-conditioning consisted of 5 minutes of lower limb ischaemia followed by 5 minutes of reperfusion using a blood-pressure cuff inflated to a pressure of 200 millimetres of mercury, also repeated three times over 30 minutes. We assayed creatine kinase-MB, troponin I.

Mean age, cardiopulmonary bypass times, and aortic cross-clamp times were matched across groups. Both post-conditioning and remote ischaemic pre-conditioning reduced the peak release of creatine kinase-MB (86.1 plus or minus 24.1 units per litre and 92.8 plus or minus 20.6 units per litre, respectively, versus 111.0 plus or minus 44.6 units per litre in the control, p less than 0.05) and troponin I (0.28 plus or minus 0.10 nanogram per millilitre and 0.26 plus or minus 0.09 nanogram per millilitre, respectively, versus 0.49 plus or minus 0.19 nanogram per millilitre in the control group, p less than 0.05).

Our study demonstrates that ischaemic post-conditioning and remote ischaemic pre-conditioning provide comparable myocardial benefit in children undergoing cold blood cardioplegic arrest.

To describe the difficulties and differing techniques in the transcatheter placement of amplatz ventricular septal defect devices to close perimembranous ventricular septal defects and place these in the context of the expanding literature on ventricular septal defect catheter closure.

Surgery remains the established first-line therapy for closure of haemodynamically significant perimembranous ventricular septal defects. Transcatheter techniques appeared to promise a possible alternative, obviating the need for cardiac surgery. However, significant technical and anatomical constraints coupled with ongoing reports of a high incidence of heart block have prevented these hopes from being realised to any significant extent. It is likely that there are important methodological reasons for the high complication rates observed. The potential advantages of transcatheter perimembranous ventricular septal defect closure over surgery warrant further exploration of differing transcatheter techniques.

Between August, 2004 and November, 2009, 21 patients had a perimembranous ventricular septal defect closed with transcatheter techniques. Of these, 14 were closed with a muscular amplatz ventricular septal defect device. The median age and weight at device placement were 8 years, ranging from 2 to 19 years, and 18.6 kilograms, ranging from 10 to 21 kilograms, respectively.

There were 25 procedures performed on 23 patients using 21 amplatz ventricular septal defect devices. Median defect size on angiography was 7.8 millimetres, ranging from 4 to 14.3 millimetres, with a median device size of 8 millimetres, ranging from 4 to 18 millimetres, and a defect/device ratio of 1.1, with a range from 0.85 to 1.33. Median procedure time was 100 minutes, with a range from 38 to 235 minutes. Adverse events included device embolisation following haemolysis in one, and new aortic incompetence in another, but there were no cases of heart block. Median follow-up was 41.7 months, with a rangefrom 2 to 71 months.

Evaluating transcatheter closure of perimembranous ventricular septal defect using amplatz ventricular septal defect devices remains important, if a technically feasible method with low and acceptable complication rates is to be identified. Incidence of heart block may be minimised by avoiding oversized devices, using muscular devices, and accepting defeat if an appropriately selected device pulls through. Given the current transcatheter technologies, the closure of perimembranous ventricular septal defects should generally be performed in children when they weigh at least 10 kilograms.

Technical developments in paediatric cardiology over the last few decades have increased expectations on professionals, demanding of them more emotional competence and communicative ability. The aim of this study was to examine the approach of paediatric cardiologists in informing and communicating with the family of the patient.

A qualitative interview method was first tested in a pilot study with two paediatric cardiologists. There were nine subsequent semi-structured interviews that were carried out with paediatric cardiologists. A researcher performed all the interviews, which were taped, transcribed, decoded, and analysed.

Among paediatric cardiologists, how to break bad news to the family is an important concern, evident in findings regarding the significance of trust and confidence, the use of different emotional positions, and a common ambition to achieve skills to handle the situation. There is a need for reflection, education, and sharing of experiences. The cardiologists desire further development of teamwork and of skills in medical students and residents for delivering bad news.

Doctors are expected to cope with the complexities of diagnoses and decisions, while simultaneously being sensitive to the feelings of the parents, aware of their own emotions, and able to keep it all under control in the context of breaking the bad news to the parents and keeping them informed. These conflicting demands create a need to expand the professional role of the doctor by including more training in emotional competence and communicative ability, beginning in medical school and continuing through consultancy.

To examine the utility of decline in arterial partial pressure of oxygen after exercise as a marker of pulmonary vascular obstructive disease in patients with atrial septal defect and pulmonary hypertension.

Treadmill exercise was performed in 18 patients with atrial septal defect and pulmonary hypertension. Arterial blood gas samples were obtained before and after peak exercise. A decline in the arterial pressure of oxygen of more than 10 millimetres of mercury after exercise was considered significant based on preliminary tests conducted on the controls. Cardiac catheterisation was performed in all patients and haemodynamic data sets were obtained on room air, oxygen, and a mixture of oxygen and nitric oxide (30–40 parts per million).

There were 10 patients who had more than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise and who had a basal pulmonary vascular resistance index of more than 7 Wood units per square metre. Out of eight patients who had less than a 10 millimetres of mercury drop in arterial partial pressure of oxygen after exercise, seven had a basal pulmonary vascular resistance index of less than 7 Wood units per square metre, p equals 0.0001. A decline in arterial partial pressure of oxygen of more than 10 millimetres of mercury predicted a basal pulmonary vascular resistance index of more than 7 Wood units per square metre with a specificity of 100% and a sensitivity of 90%.

A decline in arterial partial pressure of oxygen following exercise appears to predict a high pulmonary vascular resistance index in patients with atrial septal defect and pulmonary hypertension. This test is a useful non-invasive marker of pulmonary vascular obstructive disease in this subset.

We thought of assessing the prevalence and predictors of feeding disorders in patients with congenital heart defects after neonatal cardiac surgery.

Retrospective study of 82 consecutive neonates (48 males, 34 females) who underwent surgery for congenital heart defects from 1999 to 2002. Information was taken from patient charts and nursing notes. The presence of a feeding disorder was assessed by a questionnaire sent to the paediatricians when the child was 2 years of age. A feeding disorder was defined as a need for tube feeding, inadequate food intake for age, or failure to thrive. Data were analysed with descriptive statistics and logistic regression.

Feeding disorders occurred in 22% of the study population. Reoperation and early feeding disorders were identified as independent risk factors for later feeding disorders (odds ratio 5.8, p 0.01; odds ratio 20.7, p 0.02). There was a trend towards more feeding disorders in patients with neurological abnormalities during the first hospital stay.

Feeding disorder is a frequent, long-term sequela after neonatal cardiac surgery. Patients with congenital heart defects who undergo multiple cardiac surgeries and those with early feeding disorders are at risk of developing later feeding disorders. Patients with these risk factors need to be selected for preventive strategies.

To analyse blood pressure characteristics during 24-hour ambulatory blood pressure monitoring in children and to assess factors that influence its success over 24 hours and during patient-recorded awake (DAY) and sleep (NIGHT) periods.

A total of 169 consecutive ambulatory blood pressure monitoring studies were conducted in 154 patients over 30 months. For each ambulatory study, we measured the percentage of successful measurements both at the first attempt (S-initial%) and following any automated repeat attempt if initial attempts had failed (S-final%). These were measured over 24-hour, DAY, and NIGHT periods.

We found that blood pressure measurements at NIGHT were more successful than measurements attempted during the DAY (p<0.05). There was no influence of age, gender, height, weight, body mass index and estimated glomerular filtration rate with the proportion of successful measurements during the 24-hour, DAY, and NIGHT periods. On stepwise multiple regression analysis, the indexed mean systolic blood pressure over 24 hours was the only factor having a significant influence on the proportion of successful measurements over the 24-hour and DAY periods, although it only accounted for three-tenths of the variance; it had no influence on the overall success of measurements at NIGHT.

Ambulatory blood pressure monitoring in children provides reliable data both during the patient's awake and sleep periods with higher success of measurements at NIGHT as opposed to DAY periods.

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.

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.

To elucidate the hypothesis of ongoing inflammation in children with chronic rheumatic heart disease, and its possible consequences.

This study was conducted on 36 patients with a mean age of 12.63 years: six with acute rheumatic carditis, and 30 with chronic rheumatic heart disease. There were 15 age- and sex-matched children who served as a control group. All subjects underwent echocardiographic assessment of valvular involvement and left ventricular function. Laboratory investigations comprised lipid profile, high-sensitivity C-reactive protein, and homocysteine assay.

High-sensitivity C-reactive protein was significantly elevated in patients with acute rheumatic carditis and in patients with chronic rheumatic heart disease (mean and standard deviation of 78.33, 156 micrograms per millilitre and 78.33, 23.17 micrograms per millilitre, respectively) as compared to the control group (mean and standard deviation of 5.83 and 2.79 micrograms per millilitre). High-sensitivity C-reactive protein correlated with the grade of mitral regurgitation (p less than 0.05). Homocysteine was significantly elevated in patients with acute carditis and patients with rheumatic heart disease as compared to the control group (their mean and standard deviation were 2.96, 0.476 nanograms per decilitre, 2.99, 1.48 nanograms per decilitre, and 1.717, 0.733 nanograms per decilitre, respectively), but did not show significant difference between the two studied groups of patients. Neither C-reactive protein nor homocysteine correlated with any of the studied parameters of lipid profile.

There is evidence of ongoing inflammation in children with rheumatic heart disease, which correlates with the degree of valvular involvement. This ongoing inflammation may put those children at risk for premature atherosclerosis.

Hypertrophic cardiomyopathy is the most common cause of sudden death in young people, including trained athletes, and is caused by mutations in genes encoding proteins of the cardiac sarcomere. Mutations in the Troponin C gene (TNNC1) are a rare genetic cause of hypertrophic cardiomyopathy. We describe a novel type of mutation (c.363dupG) in Troponin C, a rare form of hypertrophic cardiomyopathy.

A family in which a 19-year-old asymptomatic male died of sudden cardiac death due to hypertrophic cardiomyopathy was genetically studied by sequencing 17 genes associated with hypertrophic cardiomyopathy or its phenocopies.

A c.363dupG mutation in Troponin C was identified, and tested across the family.

We report the first frameshift mutation (c.363dupG or p.Gln122AlafsX30) in Troponin C causing hypertrophic cardiomyopathy (and sudden cardiac death) in a 19-year-old male, and have demonstrated that the mutation segregates with hypertrophic cardiomyopathy within the family.