With improvements in the surgical and medical management of children with congenital heart disease, the majority are now expected to transition into adult services. Whilst the importance of successful transition is increasingly acknowledged, previous research has identified a number of areas of concern. We therefore wished to elicit the views of young people themselves concerning their requirements from a specialist cardiac service.

Using an exploratory approach our aims were to increase awareness of the service needs and expectations of young people, identify good practice and areas where communication processes and facilities could be improved, and enhance understanding of transition between paediatric and adult services. Postal questionnaires were sent to 63 young people with congenital cardiac disease aged 17 to 20 years. Open ended questions asked for views and experiences about facilities in the paediatric and adult areas, perceptions of transition between services and suggestions for improvements. Qualitative data were transferred verbatim into electronic format to facilitate coding and theming.

Thirty-eight (60.3 percent) completed questionnaires were received. A number of themes, including communication, care processes/caring interactions and environment, emerged regarding areas of satisfaction and dissatisfaction within the paediatric and adult services, together with areas for improvement and important aspects of transition.

Young people’s views need to be taken into account at all stages of the patient journey. They want an individualised approach to their care which recognises that they have specific emotional and practical needs. Such findings need to be incorporated into an ongoing model of care.

Several clinical and echocardiographic studies describe morphological and functional findings in patients with hypertrophic cardiomyopathy. Less is known regarding morphological and functional characteristics of the left ventricular hypertrophy found in the setting of the Noonan and LEOPARD syndromes.

To compare non-invasively the morphological and functional findings potentially affecting symptoms and clinical outcome in children with hypertrophic cardiomyopathy as opposed to Noonan and LEOPARD syndromes.

We studied by echo-Doppler 62 children with left ventricular hypertrophy, dividing them into two subgroups matched for age and body surface area. The first group, of 45 patients with a mean age of 7.5 ± 5.2 years and body surface area of 0.9 ± 0.44 mq, had idiopathic hypertrophic cardiomyopathy. The second group, of 17 patients, all had left ventricular hypertrophy in the setting of Noonan or LEOPARD syndromes. Their mean age was 6.6 ± 5 years, and body surface area was 0.8 ± 0.36 mq. In all patients, we assessed the left ventricular maximal mural thickness, expressed as a Z-score, along with any obstructions in the left and right ventricular outflow tracts. In addition, to define left ventricular diastolic function, we used mitral flow and pulsed Tissue Doppler to record the Ea, Aa, Ea/Aa, E/Ea indexes in the apical 4-chamber view at the lateral corner of the mitral annulus. We also measured the diameters of the coronary arteries in the diastolic frame.

Compared to those with hypertrophic cardiomyopathy, those with syndromic left ventricular hypertrophy showed a significantly increased Z-score for mural thickness, and a higher prevalence of obstruction in the left ventricular outflow tract. In addition, the patients with Noonan or LEOPARD syndromes showed a significantly decrease of Ea and increase of Aa, with a decreased Ea/Aa ratio, all suggestive of left ventricular abnormal relaxation. Moreover, the E/Ea ratio was significantly increased in these patients. The presence of right ventricular hypertrophy, mainly associated with dynamic obstruction in the outflow tract, was detected in only 5 of the 17 patients with Noonan or LEOPARD syndromes, as was dilation of the coronary arteries.

Compared to children with hypertrophic cardiomyopathy, those with left ventricular hypertrophy in the setting of Noonan or LEOPARD syndromes show more ventricular hypertrophy and diastolic dysfunction, due to both abnormal relaxation and reduced compliance. They also exhibit an increased prevalence of obstruction of the left ventricular outflow tract, along with dynamic obstruction of the right ventricular outflow tract and dilated coronary arteries. These morphological and functional findings could explain the different symptoms and clinical events, and potentially define the more appropriate therapeutic options in children with left ventricular hypertrophy of different aetiology.

We aimed, in this study, to compare dispersion of the p wave in patients with type 1 diabetes to nondiabetic control subjects, and to investigate the relationship between the dispersion of the p wave and cardiac autonomic dysfunction in diabetic children.

We enrolled 49 patients with type 1 diabetes, and 32 age- and sex-matched healthy subjects, measuring the Valsalva ratio, resting heart rate, and orthostatic hypotension in all. The duration of the p wave was measured manually on a high-resolution computer screen. Dispersion, defined as the difference between maximum and minimum durations of the p waves, was also measured in the 12-lead electrocardiogram before and after the Valsalva maneuver.

The mean age of the patients and their controls were 14.2 ± 4.8 years, and 12.7 ± 4.5 years, respectively. The mean duration of diabetes had been 6.2 ± 4.6 years. Maximal and minimal values for the duration of the p wave were significantly decreased in the diabetic children, with the dispersion itself significantly increased. Values for the dispersion in the diabetic subjects were similar before and after the Valsalva maneuver, whereas dispersion was found significantly increased after this maneuver in the controls. The differences in the Valsalva ratio, resting heart rate, and orthostatic hypotension between the groups, on the other hand, were not found to be statistically significant.

The noted increase in the dispersion of the p wave in diabetic children reveals the onset of cardiac electrophysiological heterogeneity before it is possible to detect parasympathetic and sympathetic dysfunction with other tests.

There is a great need for echocardiographic criterions for accurate diagnosis of carditis in acute rheumatic fever.

To test the efficacy of proposed echocardiographic criterions for the diagnosis of carditis.

We studied 333 patients suspected of having acute rheumatic fever, undertaking detailed clinical examination, laboratory tests and meticulous echocardiography in each case. We used previously established echocardiographic criterions for the diagnosis of carditis and subclinical valvitis. In 220 cases (66.06%), both the echo criterions, and the Jones’ criterions, gave positive results. In 52 cases (15.61%), we found evidence of subclinical carditis, in that clinically no murmur was heard, meaning the Jones’ criterions were negative, but the echocardiographic evaluation was positive. In 4 patients clinically diagnosed as having carditis, the Jones’ criterions were positive, but echocardiographic evaluation showed them to have congenitally malformed hearts. In another 57 cases (17.11%), the Jones’ criterions were negative, as were the results of echocardiographic evaluation. These patients were taken as control subjects. On this basis, the echocardiographic criterions had sensitivity of 81% and specificity of 93%.

Using our echocardiographic criterions, it is possible to make a precise diagnosis of carditis or subclinical valvitis. Hence, echocardiography should, in future, be included as a major criterion in the Jones’ system.

To evaluate echocardiographically the function and morphology of the right heart subsequent to transcatheter closure of atrial septal defects.

We performed echocardiographic studies in 73 patients undergoing transcatheter closure of atrial septal defects 1 day prior to closure, and then 3 days and 3 months after closure. We calculated the antero-posterior diameter of the right ventricle, the transverse diameter and length of the right atrium, the pulmonary arterial pressure, and the velocities of systolic movement and early and late diastolic movement of the basal parietal walls of the right ventricle.

The atrial septal defects varied in size between 8 and 33 millimetres, and were closed using occluders measuring from 10 to 40 millimetres. At 3 days after closure, the antero-posterior diameter of the right ventricle, the transverse diameter and length of the right atrium, the pulmonary arterial pressure, and the velocities of mural motion were all significantly decreased. After 3 months, the size of the right heart had more or less normalized.

Transcatheter closure of atrial septal defects produces marked improvement in the function and geometry of the chambers of the right heart, reducing pulmonary arterial pressure as well as abolishing the interatrial shunt.

To assess neurohormonal activation of cardiac failure in adults with congenitally corrected transposition, and to determine the most sensitive marker for recognition of the cardiac failure.

The onset of morphologically right ventricular dysfunction is unpredictable in patients with congenitally corrected transposition, the combination of discordant atrioventricular and ventriculo-arterial connections, and its markers are unknown.

We measured amino terminal pro brain natriuretic peptide in 19 patients, aged 35 plus or minus 13.1 years, and in 19 control subjects. Morhologically right ventricular function was assessed by echocardiography, including tissue Doppler echocardiography and magnetic resonance imaging or multislice computed tomography.

The patients showed a highly significant increase in the levels of amino terminal pro brain natriuretic peptide, the levels being significantly elevated even in asymptomatic patients. Left atrial dimensions were larger in patients, and significantly lower tissue Doppler echocardiographic velocities were measured at the lateral site of the tricuspid annulus and at the basal segment of the interventricular septum. The ejection fraction of the morphologically right ventricle correlated significantly with the levels of brain natriuretic peptide, and with left atrial dimensions.

Neurohormonal activation is present in patients with congenitally corrected transposition even when they are asymptomatic. It is correlated with left atrial dimensions and tissue Doppler echocardiographic parameters. Levels of brain natriuretic peptide, and peak tricuspid early diastolic annular velocity, are the earliest and most sensitive markers of morphologically right ventricular dysfunction.

The strategies of repair of tetralogy of Fallot change with the age of patients. In children older than 4 years and adults, the optimal strategy may be to use different method of reconstruction of the right ventricular outflow tract from those followed in younger children, so as to avoid, or reduce, the pulmonary insufficiency that is increasingly known to compromise right ventricular function.

From April, 2001, through May, 2008, we undertook complete repair in 312 patients, 180 male and 132 female, with a mean age of 11.3 years ±0.4 years, and a range from 4 to 48 years, with typical clinical and morphological features of tetralogy of Fallot, including 42 patients with the ventriculo-arterial connection of double outlet right ventricle. The operation was performed under moderate hypothermia using blood cardioplegia. The ventricular septal defect was closed with a Dacron patch. When it was considered necessary to resect the musculature within the right ventricular outflow tract, or perform pulmonary valvotomy, we sought to preserve the function of the pulmonary valve by protecting as far as possible the native leaflets, or creating a folded monocusp of autologous pericardium.

The repair was achieved completely through right atrium in 192, through the right ventricular outflow tract in 83, and through the right atrium, the outflow tract, and the pulmonary trunk in 36 patients. A transjunctional patch was inserted in 169 patients, non-valved in all but 9. There were no differences regarding the periods of aortic cross-clamping or cardiopulmonary bypass. Of the patients, 5 died (1.6%), with no influence noted for the transjunctional patch. Of those having a non-valved patch inserted, three-tenths had pulmonary regurgitation of various degree, while those having a valved patch had minimal pulmonary insufficiency and good right ventricular function postoperatively, this being maintained after follow-up of 8 to 24-months.

Based on our experience, we suggest that the current strategy of repair of tetralogy of Fallot in older children and adults should be based on minimizing the insertion of transjunctional patches, this being indicated only in those with very small ventriculo-pulmonary junctions. If such a patch is necessary, then steps should be taken to preserve the function of the pulmonary valve.

It is just as vital to have an exact overview of the physical fitness of young and growing people as it is for adults. The currently used exercise protocols have limitations in healthy small children, and in senior citizens. In particular with chronically ill patients, regardless of their age, there is a need for an exercise protocol that permits observations over the long term. With this need in mind, we have designed a new transferable standardised exercise protocol, constructing reference values based on improved assessments on a treadmill that permitted stepwise increases of speed and gradient every 90 seconds – the so called treadmill protocol from the German Society of Paediatric Cardiology.

We investigated the exercise performance in a healthy Caucasian population ranging in age from 4 to 75 years.

We measured, using a prospective study design, the distance run, the endurance, and the consumption of oxygen in 548 females and 647 males undergoing an enhanced spiroergometric treadmill protocol in two centres.

Until puberty, boys and girls have the same indicators of exercise performance. Subsequent to puberty, uptake of oxygen and distance run differ, with males showing higher uptake of oxygen. There is still an age-dependent dynamic of peak uptake of oxygen related to body surface area. Using these new reference values, covering the whole range of age, it proves possible to compare performance during growth and aging of the individual. In this fashion, we have calculated centiles for all recorded variables. External calibration, validation and quality control ensures transferability of our data to other spiroergometry units.