Complications at the vascular access sites are among the most common adverse events in congenital cardiac catheterization. The use of small-gauge catheters may reduce these events; however, other factors can contribute to the development of vascular complications.

To determine factors associated with the development of vascular access complications in children undergoing congenital cardiac catheterization.

We performed a prospective study of 403 patients who underwent diagnostic (62.5%) or interventional (37.5%) cardiac catheterization over a period of 6 months, and analysed the vascular complications during and immediately after the procedure.

The most common access-related adverse event was transient loss of pulsation (17.6%). Other less common access-related adverse events included subcutaneous haematoma (2%), bleeding (3%), vessel tear (0.2%), and vein thrombosis (0.2%). Patients who had no access-related adverse events had significantly higher age and body weight compared with those who had one or more access problems. Among 81 patients who had vascular access established in unplanned access sites, 30 patients (37%) had lost pulsations. Among the 322 patients who had vascular access established in planned access sites, however, only 41 patients had lost pulsation (13%). In addition, patients who had lost pulsations had significantly longer puncture time compared to those who had normal pulsations (p value 0.01).

Factors other than sheath size can contribute to access-related adverse events in children undergoing cardiac catheterization. Obtaining vascular access in unplanned access sites and longer puncture times increases the incidence of lost pulsations after catheterization. Younger age and smaller body weight are also associated with significant increase in access-related adverse events.

This study compares the developmental and functional outcomes at school entry between boys and girls born with a congenital cardiac defect who required early surgical correction.

A prospective cohort of 94 children, including 49 percent boys, were followed up to 5 years of age and assessed for developmental progress. Developmental measures included Wechsler Preschool and Primary Scale of Intelligence – cognitive; Peabody Picture Vocabulary Test – receptive language; Peabody Developmental Motor Scale – motor; and Child Behaviour Checklist – behaviour. Measures of function included the Vineland Adaptive Behavior Scale and Functional Independence Measure for Children (WeeFIM).

The mean scores of the boys on the WeeFIM subscales, such as self-care, mobility, cognition, were significantly lower than that of the girls. There was a trend for a greater proportion of boys to have abnormalities on neurological examination (boys 37.5 percent abnormal, girls 19.5 percent abnormal). Verbal, performance, and full scale Intellectual Quotients were 5–7 points lower in boys but did not reach significance (full scale Intellectual Quotient: boys 87.7 plus or minus 22.2; girls 93.9 plus or minus 19.3). Boys were more likely to have fine motor delays (50 percent, 82.7 plus or minus 16.5) compared with girls (28.2 percent, 87.0 plus or minus 15.8). There were no gender differences in receptive language or behavioural difficulties.

Boys born with congenital heart disease requiring early surgical repair appear to be at enhanced risk for neuromotor impairments and activity limitations. Findings support gender differences in the pathogenesis of early brain injury following hypoxic–ischaemic insults. This has implications for neuroprotective strategies to prevent brain injury.

Interatrial septal anomalies, which include atrial septal defect, patent foramen ovale, and atrial septal aneurysm, are common disorders among adult patients. Early detection of interatrial septal anomalies is important in order to prevent haemodynamic consequences and/or thromboembolic events. Electrocardiogram offers some clues that should serve as hints for detection of interatrial abnormalities. The aim of our study was to analyse the interatrial septum by transoesophageal echocardiography in patients with electrocardiogram signs of right bundle branch block and in those without right bundle branch block.

In a prospective study, 87 adult patients were included, that is, 41 with electrocardiogram signs of right bundle branch block forming the first group and 46 without right bundle branch block forming the second group. Interatrial septal anomalies were present in 80.5% of the patients with right bundle branch block, with patent foramen ovale (39.02%) being the most prevalent disorder, followed by atrial septal aneurysm (21.9%) and atrial septal defect (19.5%). Interatrial septal abnormalities were significantly more frequent in the first group compared with the second group (80.5% versus 6.5%, p value less than 0.001). Independently, patent foramen ovale was significantly more prevalent in patients with right bundle branch block (39.02% versus 4.3%, p value less than 0.001), as were atrial septal aneurysm (21.9% versus 2.2%, p value equal 0.01) and atrial septal defect (19.5% versus 0%, p value equal 0.004).

Right bundle branch block should serve as a valuable indicator to motivate a detailed search for interatrial septal abnormalities.

Systemic right ventricular systolic dysfunction is common late after atrial switch surgery for transposition of the great arteries. Total isovolumic time is the time that the ventricle is neither ejecting nor filling and is calculated without relying on geometric assumptions. We assessed resting total isovolumic time in this population and its relationship to exercise capacity.

A total of 40 adult patients with transposition of the great arteries after atrial switch – and 10 healthy controls – underwent transthoracic echocardiography and cardiopulmonary exercise testing from January, 2006 to January, 2009. Resting total isovolumic time was measured in seconds per minute: 60 minus total ejection time plus total filling time.

The mean age was 31.6 plus or minus 7.6 years, and 38.0% were men. There were 16 patients (40%) who had more than or equal to moderate systolic dysfunction of the right ventricle. Intra- and inter-observer agreement was good for total isovolumic time, which was significantly prolonged in patients compared with controls (12.0 plus or minus 3.9 seconds per minute versus 6.0 plus or minus 1.8 seconds per minute, p-value less than 0.001) and correlated significantly with peak oxygen consumption (r equals minus 0.63, p-value less than 0.001). The correlation strengthened (r equals minus 0.73, p-value less than 0.001) after excluding seven patients with exercise-induced cyanosis. No relationship was found between exercise capacity and right ventricular ejection fraction or long-axis amplitude.

Resting isovolumic time is prolonged after atrial switch for patients with transposition of the great arteries. It is highly reproducible and relates well to exercise capacity.

Cross-sectional two-dimensional echocardiography can establish correct diagnosis in all types of atrial communications; however, real-time three-dimensional reconstruction provides additional value to the surgeon and interventionist for better understanding of spatial intracardiac morphology.

This study was carried out to define the anatomical criteria for the diagnosis of inferior-type caval vein defect and compare the echocardiographic findings with surgical findings.

The records of 19 patients – 13 male and six female patients in the age group of 18 months to 27 years, who were diagnosed as inferior-type caval vein defect with or without anomalous drainage of right pulmonary vein(s) on echocardiography – were retrospectively reviewed and compared with surgical findings.

Surgical diagnosis of inferior-type caval vein defect was confirmed in 17 of the 19 patients. In two patients, the surgical diagnosis was that of a large fossa ovalis atrial septal defect – confluent defect and fossa ovalis atrial septal defect with deficient inferior rim in one patient each. Surgical diagnosis of anomalous drainage of pulmonary vein(s) was based on the course of the superior rim of the defect in relation to the pulmonary veins. Our echocardiographic impression of the pulmonary veins appearing in its normal position but showing abnormal drainage to right atrium was in agreement with the surgical notes. Discrepancy was found in the number of pulmonary veins draining anomalously. The discordance was related to overdiagnosis of anomalous drainage in all except one, that is, three out of four. In one, only the right lower pulmonary vein was diagnosed to be anomalous, whereas both right upper and lower pulmonary veins were found to be anomalous.

Echocardiography provides definite diagnosis of inferior-type caval vein defect. Inferior caval vein straddling and an intact fossa ovalis are prerequisites for diagnosis. Anomalous pulmonary venous drainage of the right pulmonary veins is very common in our series, although accurate diagnosis of the number of pulmonary veins was not possible in all cases. Multiple views on transthoracic echocardiography starting from the subxiphoid views delineate the morphology accurately. Transoesophageal echocardiography is required only in patients in whom the windows, especially the subxiphoid, are not adequate.

Poor weight gain is common in infants after Stage I Norwood operation and can negatively impact outcomes.

The purpose of this study was to examine the impact of feeding strategy on interstage weight gain.

In a multi-centre study, 158 infants discharged following the Norwood operation were enrolled prospectively. Weight and feeding data were obtained at 2-week intervals. Differences between feeding regimens in average daily weight gain and change in weight-for-age z-score between Stage I discharge and Stage II surgery were examined.

Discharge feeding regimens were oral only in 52%, oral with tube supplementation in 33%, and by nasogastric/gastrostomy tube only in 15%. There were significant differences in the average daily interstage weight gain among the feeding groups – oral only 25.0 grams per day, oral/tube 21.4 grams per day, and tube only 22.3 grams per day – p = 0.019. Tube-only-fed infants were significantly older at Stage II (p = 0.004) and had a significantly greater change in weight-for-age z-score (p = 0.007). The overall rate of weight gain was 16–32 grams per day, similar to infant norms. The rate of weight gain declined over time, with earlier decline observed for oral- and oral/tube-fed infants (less than 15 grams per day at 5.4 months) in comparison with tube-only-fed infants (less than 15 grams per day at 8.6 months).

Following Stage I Norwood, infants discharged on oral feeding had better average daily weight gain than infants with tube-assisted feeding. The overall weight gain was within the normal limits in all feeding groups, but the rate of weight gain decreased over time, with an earlier decline in infants fed orally.

To assess the reference values of M-mode echocardiographic measurements in healthy newborns according to body weight.

From January, 2008 to June, 2008, a total of 250 – 130 female and 120 male – term healthy newborns were included in the study. M-mode echocardiographic measurements were recorded by a paediatric cardiologist in accordance with the American Society of Echocardiography recommendations. Right ventricular anterior wall end-diastolic thickness, right ventricular end-diastolic diameter, interventricular septum end-diastolic thickness, left ventricular end-diastolic diameter, left ventricular posterior wall end-diastolic thickness, interventricular septum end-systolic thickness, left ventricular end-systolic diameter, left ventricular posterior wall end-systolic thickness, aortic root dimension, left atrium dimension, left ventricular ejection, and shortening fraction were measured.

The values of echocardiographic measurements revealed a good correlation with body weight. When body weight increased, the measured values also increased in parallel. The measured values were not influenced by gender. Ejection and shortening fraction parameters did not change with body weight and gender.

In this study, normative values related to body weight for cardiac chambers and wall thickness were determined in healthy newborns. These reference values can be used to evaluate whether newborns have normal or abnormal echocardiographic measurement.

The objective of this study was to compare three patient-based New York Heart Association assessments with cardiologist assessments in outpatients with congenital cardiac disease.

Consecutive adult outpatients completed three questionnaires in a random order: a patient-based translation of the New York Heart Association classes, a self-constructed questionnaire based on the New York Heart Association classes, and the Specific Activity Scale. The treating cardiologist assessed the New York Heart Association class on the same day. Patient–cardiologist agreement was assessed by calculating percent agreement and weighted kappa. We also explored the level of agreement for patients without co-morbidity.

In all, 86 adults – with a median age of 35.8 years – including 46 women participated. An agreement of 75.6% (weighted kappa is 0.43; probability is smaller than 0.01), 70.6% (weighted kappa is 0.44; probability is smaller than 0.01), and 74.4% (weighted kappa is 0.28; probability is smaller than 0.01) was found between the cardiologist assessment and the patient-based translation, self-constructed questionnaire, and the Specific Activity Scale, respectively. The patient-based translation equally over- and underestimated the New York Heart Association class, whereas the self-constructed questionnaire overestimated and the Specific Activity Scale underestimated the New York Heart Association class. Agreement levels for patients without co-morbidity were higher than agreement levels for the total group.

The patient-based translation yielded adequate agreement with cardiologist-assessed New York Heart Association class, showed equal over- and underestimation, and was easy to complete. The patient-based translation with the instruction to only consider functional impairments caused by the congenital cardiac defect is recommended in future studies of outpatients with congenital cardiac disease.

To describe the experiences of children and adolescents being screened positive for hypertrophic cardiomyopathy and how this impacts their daily life.

Hypertrophic cardiomyopathy is a hereditary disease and the most common medical cause of sudden death in childhood and adolescence. This is the reason for recommending screening in children with an affected first-degree relative. A diagnosis of hypertrophic cardiomyopathy implies lifestyle modifications, restrictions that may bring profound changes to the daily life of the affected individual.

This is a descriptive qualitative interview study.

We interviewed 13 asymptomatic children or adolescents diagnosed with hypertrophic cardiomyopathy through family screening 12–24 months after the diagnosis. Analysis was conducted with qualitative content analysis.

Children described an involuntary change, which affected their daily life with limitations and restrictions in life, both in the individual and social context. Lifestyle recommendations had the most severe impact on daily life and affected their social context. They tried to navigate in a world with new references, and after reorientation they felt hope and had faith in the future.

Children diagnosed with hypertrophic cardiomyopathy through family screening went through an involuntary change resulting in limitations and restrictions in life. This study indicates that there is a need for support and that healthcare professionals have to consider the specific needs in these families. Our findings thus give guidance in how best to improve support to the patients and their family. Diagnosis in asymptomatic children should be accompanied by ideally multi-professional follow-up, focusing not only on medical issues.

We performed a retrospective analysis of patients with pulmonary arterial hypertension receiving inhaled iloprost in a single centre to evaluate long-term tolerability, safety, and efficacy of chronic inhaled iloprost therapy in children.

A total of 20 patients with either idiopathic or associated pulmonary arterial hypertension were treated with iloprost between April, 2003 and January, 2010. The median age and weight of the patients were 3.8 years – ranging from 4 months to 19 years – and 12.3 kilograms – ranging from 4 to 73 kilograms – respectively. Pulmonary arterial hypertension was idiopathic or hereditary in eight patients (40%) and associated with congenital cardiac disease in 12 patients (60%).

Of the 20 patients, 15 had combined therapy – 12 patients with two and three patients with three different classes of drugs. In all, six patients died during follow-up. The median follow-up time was 18 months, ranging from 6 to 74 months. The 6-minute walking test was performed in 7 out of 20 patients at baseline and on follow-up. The median 6-minute walking test increased from 420 to 490 metres after iloprost therapy (p = 0.028). After initiation of iloprost therapy, one patient complained of headache and another had a rash around his mouth, none necessitating discontinuation of therapy. Overall compliance with inhaled iloprost was good.

Pulmonary hypertension is associated with significant morbidity and mortality. Careful assessment of each patient and timely combination of specific vasodilator therapy is needed to improve clinical outcomes. This study suggests that inhaled iloprost, with or without concomitant endotelin receptor antagonist and/or phosphodiesterase inhibitor, is safe and efficacious for treatment of pulmonary arterial hypertension in children.

Atomoxetine is a central norepinephrine reuptake inhibitor used to treat attention deficit/hyperactivity disorder. The effects of atomoxetine on cardiovascular functions and QT dispersion in children with attention deficit/hyperactivity disorder have not been previously reported. The aim of this study was to analyse cardiovascular functions and QT dispersion on the surface electrocardiogram of children with attention deficit/hyperactivity disorder during atomoxetine therapy.

A total of 40 children – with a mean age of 8.6 plus or minus 2.3 years and a median age of 11 years; ranged from 8 to 14 years – with attention deficit/hyperactivity disorder – with six girls and 34 boys – were included in the study. We recorded the mean systolic and diastolic blood pressure, heart rate, corrected QT interval, QT dispersion, and left ventricular systolic functions at baseline and 5 weeks after atomoxetine therapy.

Atomoxetine decreased baseline mean systolic and diastolic blood pressure; baseline mean heart rate decreased; and baseline mean corrected QT interval and QT dispersion mildly increased. Atomoxetine decreased baseline mean ejection fraction and baseline mean shortening fraction.

The results of our study suggest that atomoxetine does not cause clinically significant alterations in QT dispersion, systolic and diastolic blood pressure, heart rate, corrected QT interval, and left ventricular systolic functions during short-term treatment in children with attention deficit/hyperactivity disorder.

Acute rheumatic fever is a systemic inflammatory disease occurring after acute streptococcal tonsillopharyngitis. The PR prolongation in these patients is thought to be due to increased vagal activity. There has been no previous study investigating the autonomic balance in patients with acute rheumatic fever. In this study, we aimed to investigate the balance of the autonomic nervous system in children with acute rheumatic fever by analysis of heart rate variability.

We evaluated the heart rate variability parameters in 50 patients with acute rheumatic fever and 37 comparable control subjects. Both groups underwent 24-hour electrocardiography monitoring, and time- and frequency-domain heart rate variability parameters were calculated. A total of 39 patients (78%), with (n = 28) or without (n = 11) other major findings, had carditis, and the remaining 11 (22%) did not. The PR interval was found to be prolonged in 10 (20%) of the patients at the beginning.

In the study group, the time- and frequency-domain heart rate variability parameters showed a sympathetic dominance compared with the control group, with a p-value less than 0.05. When compared with the control group, the time- and frequency-domain heart rate variability parameters showed a significant sympathetic dominance in patients with both prolonged PR and normal PR intervals in the acute period, with a p-value less than 0.05. When compared with patients with normal PR interval, mean normalised low frequency and normalised high frequency parameters suggested a relatively lower sympathetic dominance in patients with prolonged PR interval, with a p-value less than 0.05.

Our results indicated that in the acute period of rheumatic fever, sympathetic dominance is apparent; in patients with prolonged PR interval, sympathetic dominance is relatively lower when compared with the patients with normal PR interval.

To objectively evaluate and describe physical activity levels in children with a stable congenital heart defect and compare those levels with children who do not have a congenital heart defect.

We matched 21 pairs of children for gender and grade in school and gave them an accelerometer-based motion sensor to wear for 7 consecutive days.

Physical activity levels did not differ between children with and without a congenital heart defect. During the 7 days of monitoring, children in this study spent most of their time in sedentary behaviours, that is, 6.7 hours of the 13 monitored hours, 54 minutes in moderate-intensity physical activity, and 12 minutes in vigorous-intensity physical activity. Less than one-fifth of all participants, with or without a congenital heart defect, accumulated sufficient physical activity to meet current physical activity recommendations for children and adolescents.

Children with a stable congenital heart defect have activity behaviours that are similar to children without a congenital heart defect. Habitual physical activity in children with a congenital heart defect should be encouraged early on in life to develop strong physical activity habits that will hopefully follow them across their lifespan.