Although sports participation is allowed to most adult patients with corrected tetralogy of Fallot, a reduced exercise tolerance and reduced perceived physical functioning is often present in these patients. We aimed to investigate daily physical activity in adults with tetralogy of Fallot and to investigate the underlying determinants of physical activity in daily life.

We studied 73 patients with tetralogy of Fallot (53 male; mean age 27.3 ± 7.9 years) who underwent echocardiography and cardiopulmonary exercise testing, and who completed questionnaires about physical activity and perceived health status. All variables were compared with data from a general population. Relationships were studied by Pearson or Spearman correlation coefficients with correction for multiple testing.

Patients were significantly less active compared with the general population (p > 0.05), 55% of all patients were sedentary, 27% had an active or moderately active lifestyle, and 18% of the group had a vigorously active lifestyle. Peak oxygen uptake (71 ± 16%; p < 0.0001) was significantly reduced and related to reduced physical activity levels (r = 0.229; p = 0.017) and perceived physical functioning (r = 0.361; p = 0.002).

Adult patients with tetralogy of Fallot have a sedentary lifestyle and are less active than the general population. Inactivity significantly contributes to reduced exercise capacity, in addition to the impairment based on the cardiac condition. Moreover, reduced exercise capacity and the intensity of sports performed in daily life are related to perceived physical functioning. Individual patient counselling on physical activity might be a low-cost, high-benefit measure to be taken in this patient population.

There may be an increase in the risk of atrial arrhythmia due to left atrial enlargement and the influence on conduction system in acute rheumatic fever. The aim of this study is to investigate atrial electromechanical delay and P-wave dispersion in patients with acute rheumatic fever.

A total of 48 patients diagnosed with acute rheumatic fever and 40 volunteers of similar age, sex, and body mass index were included in the study. The study groups were compared for M-mode echocardiographic parameters, interatrial electromechanical delay, intra-atrial electromechanical delay, and P-wave dispersion.

Maximum P-wave duration, P-wave dispersion, and interatrial electromechanical delay were significantly higher in patients with acute rheumatic fever compared with the control group (p < 0.001). However, there was no difference in terms of intra-atrial electromechanical delay (p > 0.05). For patients with acute rheumatic fever, a positive correlation was identified between the left atrium diameter and the P-wave dispersion and interatrial electromechanical delay (r = 0.524 and p < 0.001, and r = 0.351 and p = 0.014, respectively). Furthermore, an important correlation was also identified between the P-wave dispersion and the interatrial electromechanical delay (r = 0.494 and p < 0.001).

This study shows the prolongation of P-wave dispersion and interatrial electromechanical delay in acute rheumatic fever. Left atrial enlargement can be one of the underlying reasons for the increase in P-wave dispersion and interatrial electromechanical delay.

We aimed to assess cancer risk in congenital heart defect patients, with and without Down's syndrome, compared with the general population.

We identified all patients born and diagnosed with congenital heart defects from 1977 to 2008 using the Danish National Registry of Patients, covering all Danish hospitals. We compared cancer incidence in the congenital heart defect cohort with that expected in the general population (∼5.5 million) using the Danish Cancer Registry, and computed age- and gender-standardised incidence ratios.

We identified 15,905 congenital heart defect patients, contributing a total of 151,172 person-years at risk; the maximum length of follow-up was 31 years (median 8 years). In all, 53 patients were diagnosed with cancer, including 30 female and 23 male patients (standardised incidence ratio = 1.63; 95% confidence interval: 1.22–2.13). Risks were increased for leukaemia, brain tumours, and basal cell carcinoma. After excluding 801 patients with Down's syndrome, the standardised incidence ratio was 1.19 (95% confidence interval: 0.84–1.64). In the subgroup of 5660 non-Down's syndrome patients undergoing cardiac surgery or catheter-based interventions, the standardised incidence ratio was 1.45 (95% confidence interval: 0.86–2.29).

The overall risk of cancer among congenital heart defect patients without Down's syndrome was not statistically significantly elevated. Cancer risk in the congenital heart defect cohort as a whole, including patients with Down's syndrome, was increased compared with the general population, although the absolute risk was low. Studies with longer follow-up and more information on radiation doses are needed to further examine a potential cancer risk associated with diagnostic radiation exposure.

Flow profiles are important determinants of fluid–vessel wall interactions. The aim of this study was to assess blood flow profiles in the aorta and pulmonary trunk in patients with transposition and different ventriculoarterial connection, and hence different mechanics of the coherent pump.

In all, 29 patients with operated transposition – concordant atrioventricular and discordant ventriculoarterial connection, and no other cardiac malformation – and eight healthy volunteers were assessed with cardiac magnetic resonance imaging: n = 17 patients after atrial redirection, with a morphologic right ventricle acting as systemic pump and a morphologic left ventricle connected to the pulmonary trunk, and n = 12 patients after the arterial switch procedure, with physiologic ventriculoarterial connections. Flow-sensitive four-dimensional velocity-encoded magnetic resonance imaging was used to analyse systolic flow patterns in the aorta and pulmonary trunk, relating to helical flow and vortex formation.

In the aorta, overall helicity was present in healthy volunteers, but it was absent in all patients independent on the operation technique. Partial helices were observed in the ascending aorta of 58% of patients after arterial switch. In the pulmonary trunk, mostly parallel flow was seen in healthy volunteers and in patients after arterial switch, whereas vortex formation was present in 88% of patients after atrial redirection.

Blood flow patterns differ substantially between the groups. In addition to varying mechanics of the coherent pumping ventricles, the absent overall helicity in all patients might be explained by the missing looping of the aorta in transposition.

The purpose of this paper is to report our 10 years of experience of interventional treatment of patients with hypoplastic left heart syndrome and to focus on the frequency, type, and results of percutaneous interventions during all the stages of palliation, considering the different techniques, devices, and complications.

Constant progress in surgical treatment of congenital heart defects in the last decade has significantly improved the prognosis for children with hypoplastic left heart syndrome. However, morbidity and mortality remain relatively high. Modern interventional procedures complement or occasionally replace surgical treatment.

Between January, 2001 and December, 2010, 161 percutaneous interventions were performed in 88 patients with hypoplastic left heart syndrome. Patients were divided into four groups: (a) before the first surgical treatment including hybrid approach, (b) after first-stage Norwood operation, (c) after second-stage bidirectional Glenn operation, and (d) after third-stage Fontan operation.

Percutaneous interventions resulted in statistically significant changes in pulmonary artery pressures, vessel diameters, and O2 saturation. Complications occurred in 4.3% of interventions and were related mainly to stent implantation in stenosed pulmonary arteries.

Percutaneous interventions may result in haemodynamic stability and reduction in the number of operations. They may result in significant changes in pulmonary artery pressures, vessel diameters, O2 saturation, with a low rate of complications, which are mainly related to stent implantation in the pulmonary arteries.

The objective of the study was to identify incidence, aetiology, and outcomes of extubation failure in infants with shunt-dependent pulmonary blood flow at a single tertiary care, academic children's hospital. The second objective of this study was to determine the haemodynamic effects of transition of positive pressure ventilation to spontaneous breathing in infants with extubation failure.

Extubation failure for our study was defined as the need for positive pressure ventilation within 96 hours after extubation. We collected demographics, pre-operative, intra-operative, post-operative, and peri-extubation data in a retrospective, observational format in patients who underwent a modified Blalock–Taussig shunt between January, 2005 and March, 2011. Infants undergoing Norwood operation or Damus–Kaye–Stansel with modified Blalock–Taussig shunt were excluded from the study. The cardiorespiratory variables collected before extubation and immediately after extubation included heart rate, respiratory rate, mean arterial blood pressure, central venous pressures, near infrared spectroscopy, oxygen saturations, and lactate levels. Clinical outcomes evaluated included the success or failure of extubation, cardiovascular intensive care unit length of stay, hospital length of stay, and mortality. Descriptive and univariate statistics were utilised to compare groups with extubation failure and extubation success.

Of the 55 eligible patients during the study period, extubation failure occurred in 27% (15/55) of the patients. Of the 15 patients with extubation failure, 10 patients needed reintubation and five patients received continuous positive pressure ventilation without getting reintubated. There were three patients who had extubation failure in the first 2 hours after extubation, nine patients in the 2–24-hour period, and three patients in the 24–96-hour period. In all, eight patients were extubated in the second attempt after the first extubation failure, with a median duration of mechanical ventilation of 2 days (1 day, 6 days). The median age of patients at extubation was 19 days (12 days, 22 days) and median weight of patients was 3.6 kg (3.02 kg, 4.26 kg). In all, 38% (21/55) of the patients were intubated before surgery. The most common risk factors for failed extubation were lung disease in 46% (7/15), cardiac dysfunction in 26% (4/15), diaphragmatic paralysis in 13% (2/15), airway oedema in 6% (1/15), and vocal cord paralysis in 6% (1/15). The median duration of mechanical ventilation was 4 days (1 day, 10.5 days), median cardiovascular intensive care unit length of stay was 11 days (6.5 days, 23.5 days), and the median hospital length of stay was 30 days (14 days, 48 days). The overall mortality at the time of hospital discharge was 7%.

Extubation failure in infants with shunt-dependent pulmonary blood flow and univentricular physiology is high and aetiology is diverse. Cardiopulmonary effects of removal of positive pressure ventilation are more pronounced in children with extubation failure and include escalation in the need for oxygen requirement and increase in mean arterial blood pressure. The majority of extubation failures in this select patient population occurs in the first 24 hours. Extubation failure in these patients is not associated with increased hospital length of stay or mortality.

The management of patients with Fontan physiology who undergo scoliosis surgery is difficult. The purpose of this article was to describe our experience in the management of patients with Fontan circulation undergoing spinal surgery for correction of scoliosis.

This was a retrospective study including patients with Fontan physiology who underwent spinal orthopaedic surgery. Anaesthetic management, post-operative complications, paediatric intensive care unit and total hospital stay, and the need for blood transfusions were analysed.

We identified eight children with Fontan physiology who had undergone spinal surgery from 2000 to 2010. All patients were receiving cardiac medications at the time of spinal surgery. The mean age at surgery was 14.8 years (range 12–21). In all, three patients needed inotropic support with dopamine (3, 5, and 8 μg/kg/min), which was started during surgery. During the immediate post-operative period, one patient died because of hypovolaemic shock caused by massive bleeding and dysrythmia. Mean blood loss during the post-operative period was 22.2 cc/kg (7.8–44.6). Surgical drainages were maintained for a mean time of 3 days (range 1–7). The mean hospital stay was 9.2 days (range 6–19). Pleural effusions developed in two patients. On follow-up, one patient presented with thoracic pseudarthrosis and another with a serohaematoma of the surgical wound.

Spinal surgery in patients with Fontan circulation is a high-risk operation. These patients must be managed by a specialised team.

Patients with repaired tetralogy of Fallot have good long-term survival but less is known about the subjectively assessed quality of life or objectively measured functional status of those who have not required subsequent pulmonary valve replacement. We assessed these parameters in a group of children and adults free from pulmonary valve replacement after tetralogy of Fallot repair.

A random sample of 50 subjects – 16 children and 34 adults, aged 4.1–56.7 years – who had undergone tetralogy of Fallot repair and were free from subsequent pulmonary valve replacement underwent cardiopulmonary exercise testing and completed standardised questionnaires assessing health-related quality of life and resilience. Patients were generally asymptomatic (median New York Heart Association class = I). Exercise capacity was within two standard deviations of normal for most children and adults (mean z VO2max: 0.20 ± 1.5; mean z VE/VCO2: −0.9 ± 1.3). Children reported a total health-related quality of life score similar to healthy norms (78 ± 10 versus 84 ± 1, p = 0.73). Adult survivors also reported quality of life scores comparable to healthy norms. Resilience was highly correlated with all domains of health-related quality of life (r = 0.713, p < 0.0001).

Patients who have undergone tetralogy of Fallot repair in childhood and have not required pulmonary valve replacement have a good long-term health-related quality of life. The finding that patients with greater resilience had better health-related quality of life suggests that it may be beneficial to implement interventions to foster resilience.

Recent studies have revealed that atherosclerosis progresses faster than expected in young adults with a history of Kawasaki disease. However, it is unclear as to when these arterial changes become measurable. In this study, we evaluated subclinical arterial stiffness in young children with a history of Kawasaki disease using two-dimensional ultrasound speckle tracking.

A total of 75 children with a history of Kawasaki disease (mean age, 8.2 ± 2.8 years) and 50 healthy controls (mean age 8.3 ± 3.5 years) were included. The two regions of interest for speckle tracking were manually positioned at the anterior and posterior carotid arterial wall using a Philips iE33 (Philips Medical Systems, Bothell, WA, USA). The peak systolic strain, time to peak systolic strain, early systolic strain rate, and late systolic strain rate were continuously monitored between the two regions of interest. Furthermore, the intimal-medial thickness, stiffness β, and pressure-elastic modulus, as conventional measures of arterial stiffness, were concurrently obtained.

The peak systolic strain and late systolic strain rate differed significantly between the patients with Kawasaki disease and controls (6.69% versus 8.60%, p < 0.01, and −0.28/second versus −0.51/second, p < 0.0001, respectively). There was no difference in the time to peak systolic strain, early systolic strain rate, and conventional measures.

The arteries of patients with Kawasaki disease appear to develop mild sclerotic changes shortly after the onset of the disease.

Reduced long-axis shortening despite enhanced global function has been reported in aortic stenosis. We sought to improve the understanding of this phenomenon using multi-dimensional strain analysis in conjunction with the evaluation of left ventricular rotation and twist – ventricular torsion – using tissue Doppler techniques.

A total of 57 patients with variable severity of aortic stenosis, aortic regurgitation, or mixed aortic valve disease, subdivided into six groups, were studied. Ventricular morphology was assessed using long-axis/short-axis and mass/volume ratios, afterload using end-systolic meridional wall stress, and global performance using ejection fraction. The circumferential and longitudinal strain was measured from two-dimensional images, and left ventricular rotation and twist were estimated as the difference in rotation between the base and apex of the ventricle.

Aortic stenosis was associated with higher mass/volume, ejection fraction, circumferential strain and left ventricular rotation and twist, significantly lower end-systolic wall stress, and a trend towards lower longitudinal strain compared with normal. Myocardial mechanics in aortic regurgitation were normal despite ventricular dilation. Mixed aortic valve disease showed findings similar to aortic stenosis. Left ventricular rotation and twist correlated with midwall circumferential strain (r = 0.62 and p < 0.0001), endocardial circumferential strain (r = 0.61 and p < 0.0001), and end-systolic wall stress (r = 0.48 and p < 0.0001), but not with longitudinal strain (r = 0.18 and p > 0.05).

Myocardial mechanics are normal in patients with aortic regurgitation, independent of abnormalities in cardiac geometry. Conversely, in aortic stenosis and mixed aortic valve disease, significant alterations in the patterns of fibre shortening are found. The effects of stenosis on cardiac function seem to dominate the effect of ventricular remodelling.

The aim of this study was to evaluate the surgical treatment of complex aortic coarctation using partial cardiopulmonary bypass to increase the spinal cord protection.

A total of 15 patients (age range from 7 to 48 years) underwent coarctation repair through a left posterolateral thoracotomy with cardiopulmonary bypass. Cannulation was performed via the descending aorta and the main pulmonary artery. In all, six surgeries were performed under hypothermic circulatory arrest and nine repairs were performed under mild hypothermia. The clinical outcome regarding the development of restenosis, as well as major neurologic complication, was studied.

There was no mortality. None of the patients developed paraplegia. Of the 15 patients, two developed a recurrent stenosis at the proximal anastomosis between the aortic arch and the aortic prothesis at a mean follow-up of 5.5 years. In the remaining 13 patients, echocardiography and magnetic resonance imaging showed no evidence of a significant gradient.

Complex aortic coarctation without hypoplasia of the proximal aortic arch and intra-cardiac anomalies can be repaired with low mortality and neurologic morbidity via a left thoracotomy using cardiopulmonary bypass. The use of cardiopulmonary bypass goes along with a low risk of spinal cord and lower body ischaemia and provides a sufficient amount of time for the anastomoses.

Potential side effects of stimulants for attention deficit disorder are in the focus of scientific discussions, intensified by the higher number of prescriptions. Children with known arrhythmias or other severe cardiac problems should not receive stimulants because of their sympathomimetic effects.

This is a retrospective analysis of 24-hour Holter electrocardiograms from 100 consecutive children with attention deficit disorder from January, 2006 to April, 2012.

In all, nine children had significant ventricular arrhythmia (mean age 11.4 ± 3.1 years, 77% male, 77% received methylphenidate). All these children had ventricular parasystole – four of them with an accelerated idioventricular rhythm. A significant circadian rhythm of premature ventricular contractions in seven children and the effect of standing and exercise clearly indicate the influence of the autonomic nervous system. In these children, hourly analysis of circadian rhythm within a 24-hour period showed a highly significant correlation between premature ventricular contractions and the vagal tone indicated by the heart rate variability parameter RMSSD (r = −0.83; p < 0.001). Ventricular arrhythmia was unaffected in seven children who received methylphenidate before diagnosis and decreased during metoprolol treatment in two children.

By Holter electrocardiogram analysis, we observed a remarkably high incidence of ventricular parasystole and accelerated idioventricular rhythm in nine of 100 children with attention deficit disorder, which depends on autonomic imbalance and not on stimulant treatment.

Advances in overall management have led to an increasing number of adolescents with congenital heart disease reaching adulthood. This study aimed to evaluate the health-related quality of life in adolescents with heart disease, and examine its relationship with the adolescents’ knowledge and understanding of their congenital heart disease, its severity, and its relationship to the degree of anxiety and depression, feeling of optimism and sense of coherence experienced by the adolescents together with their social support.

Adolescents with heart disease were recruited from an ambulatory setting at a tertiary centre. Patients completed self-report questionnaires including the Paediatric Quality of Life Inventory 3.0-Cardiac Module, a questionnaire assessing the adolescents’ knowledge of their cardiac condition, the Hospital Anxiety and Depression Scale, Multidimensional Scale of Perceived Social Support, Life Orientation Test-Revised, and Sense of Coherence-13, supplemented by clinical information provided by the attending cardiologists. A total of 114 patients aged 12–20 years were recruited over 15 months. In all, 98% of patients were in New York Heart Association class I. Their health-related quality of life was found to positively correlate with a low level of anxiety and depression (Pearson correlation, r = −0.57, p < 0.001), a good knowledge of their cardiac condition (r = 0.31, p < 0.01), feelings of optimism (r = 0.39, p < 0.001), adequate social support (r = 0.27, p < 0.01), and a strong sense of coherence (r = 0.24, p < 0.01).

Adolescents’ knowledge and understanding of their cardiac abnormality together with an improved sense of well-being had a positive influence on their health-related quality of life.