Objectives: To describe the echocardiographic features, underlying causes, and outcome of fetuses with dilated cardiomyopathy. Design: A retrospective observational study between 1983 and 2003 at a tertiary centre for fetal cardiology. Patients: Affected fetuses were identified using a computerised database. We included fetuses with dilation and reduced systolic function of either the right ventricle, left ventricle, or both. We excluded fetuses with abnormal cardiac connections, arrhythmias, or stenosis of the aortic or pulmonary valves. In all, we identified 50 fetuses, born to 46 mothers. Of the fetuses, 24 had biventricular cardiomyopathy, 17 had isolated right ventricular cardiomyopathy, and 9 had isolated left ventricular cardiomyopathy. Two-thirds of the fetuses (32) were hydropic at some point during gestation. Main outcomes: A cause of cardiomyopathy was identified in 37 cases (74 per cent). This was genetic or metabolic in 11 fetuses; infective in 11; fetal anaemia, without proven parvovirus infection, in 5; of cardiac origin in 5; and an association with renal disease in 5. In 10 cases (20 per cent), the pregnancy was terminated. Based on an intention to treat, the survival to delivery was 25 of 40 (62.5 per cent, 95 per cent confidence intervals from 46 to 77 per cent), at 28 days was 17 of 40 (42.5 per cent, 95 per cent confidence intervals from 27 to 59 per cent), and at 1 year was 15 of 40 (37.5 per cent, 95 per cent confidence intervals from 23 to 54 per cent). The overall survival of non-hydropic fetuses was 9 of 18 (50 per cent), compared to 6 of 32 (18 per cent) hydropic fetuses. Conclusions: Genetic, metabolic, infective, and cardiac diseases may present with dilated cardiomyopathy during fetal life. There is a high rate of spontaneous intra-uterine and early neonatal death. The prognosis is particularly poor for hydropic fetuses.

Objective: To examine the degree to which children, considered to be at risk from infective endocarditis, had received professional education and preventive procedures in regard to dental health, and to evaluate the knowledge of their parents of the link between oral health and infective endocarditis. Materials and methods: Questionnaires were distributed to the families of 38 children under the care of paediatric cardiology. A short dental examination was carried out. Parents were asked if they knew why oral health was of particular importance in their child. Results: Of the children, 58% demonstrated evidence of previous or current dental disease, with 24% having had at least one filling, 13% with one or more teeth showing deficiency of enamel, and 39% with untreated dental caries. Only 79% of the children were registered with a dentist. According to Chi squared test, there was no difference in the dental health of registered and non registered children. Of the study group, 29% had received instruction in oral hygiene, 42% had received dietary advice, 13% had received advice regarding fluoride supplementation or had had fluoride professionally applied, and 8% had had fissure sealants. These percentages remained relatively low even if only registered children, or only registered children with previous or current dental disease, were considered. Only 64% of parents were aware of the link between the oral health of their children and infective endocarditis. Parents of children who were registered were more likely to be aware of this link than parents of children who were not registered. Conclusions: In spite of being registered with general dental practitioners, few children with congenital heart disease had received basic education in dental hygiene. Even children known to have had dental disease and, therefore, considered to be more vulnerable, were overlooked.

Objectives: To establish the prevalence of coronary arterial abnormalities in mid-trimester fetuses with pulmonary atresia with intact ventricular septum, and whether their presence correlates with right ventricular morphology. Background: The presence of coronary arterial fistulas significantly alters the surgical options and prognosis for patients with pulmonary atresia with intact ventricular septum. The lesion can reliably be diagnosed using fetal echocardiography, and further definition of the prognosis is important for counselling parents. Methods: We examined the hearts of 39 pathological specimens diagnosed during fetal life, 3 of whom died postnatally. Coronary arterial abnormalities were defined as non-connection of the left or right coronary arteries to the aorta, ostial stenosis, marked tortuosity, dilation, thickening or abnormal myocardial branching. Mild tortuosity, or myocardial bridging, were considered normal. We measured the dimensions of the tricuspid valve along with the inlet and outlet portions of the ventricles. Ebstein's malformation, tricuspid valvar dysplasia, and the presence or absence of the infundibulum, were especially noted. We examined also 12 normal hearts as controls. Results: Coronary arterial abnormalities were found in 14/39 (36%). The dimensions of the right ventricle and tricuspid valves, and the gestational ages of the fetuses, were compared for these 14 with the 25 having no abnormalities using independent t-tests. The gestational ages were similar, 21.9 vs 21.1 weeks. The mean dimensions of the tricuspid valve, median z-scores, and right ventricle were smaller, 2.9 vs 7.2 mm; p < 0.002; −4.46 vs 0.23; p < 0.03; and 6.9 vs 13.7 mm; p < 0.002, for those with coronary arterial abnormalities. Ebstein's malformation, or dysplasia of the tricuspid valve, was present in 4 of 14 with, vs 15 of 25 without, coronary arterial abnormalities. A patent infundibulum was noted in 34 of 39 specimens. Conclusions: Mid-trimester fetuses with pulmonary atresia with intact ventricular septum already exhibit coronary arterial abnormalities, with a prevalence of 36%. The presence of a patent infundibulum confirms that atresia of the pulmonary valve is an acquired process. Coronary arterial abnormalities are seen in 50% of those with hypoplastic right ventricles, but less frequently in the presence of well developed ventricles. This is important information for those involved in counselling parents.

Between 1989 and 2000, 21 fetuses were diagnosed with complete atrioventricular block. Seven women with fetal ventricular rates of less than 60 were given oral terbutaline, and 6 of these had an initial increase in the fetal ventricular rate. Four fetuses (57%) maintained an increased average rate of 60 beats per minute and survived. Two fetuses returned to rates below 55 and died. The final fetus, with hypertrophic cardiomyopathy, was unresponsive. Terbutaline, therefore, is initially effective in raising the fetal ventricular rate, but this effect may be transient.

The Fontan circuit is characterized by a pulmonary circulation dependent on central venous pressure in the absence of a subpulmonary ventricle. Patients with this circulation usually exhibit a low cardiac output. In addition to their abnormal cardiac state, recent studies have demonstrated impaired cardiac autonomic nervous activity, elevated neurohormonal factors, and impaired endothelial function, findings also encountered in adults with chronic heart failure. One major component of these impairments is the abnormal cardiorespiratory response during exercise. Despite some pathophysiologic similarities to adults with cardiac disease, those with the Fontan circulation usually have a congenitally malformed heart underscoring abnormal haemodynamics, with further compromise from multiple surgical procedures. It differs in many aspects from the situation in the adults with impaired systolic ventricular function in the setting of chronic cardiac failure. In this review, I will focus on the cardiorespiratory response during exercise, seeking to clarify the characteristics in these particular patients with the Fontan circulation.

Objective: To evaluate the role of the concentration of brain natriuretic peptide in the plasma, and its correlation with haemodynamic right ventricular parameters, in children with overload of the right ventricle due to congenital cardiac disease. Methods: We studied 31 children, with a mean age of 4.8 years, with volume or pressure overload of the right ventricle caused by congenital cardiac disease. Of the patients, 19 had undergone surgical biventricular correction of tetralogy of Fallot, 11 with pulmonary stenosis and 8 with pulmonary atresia, and 12 patients were studied prior to operations, 7 with atrial septal defects and 5 with anomalous pulmonary venous connections. We measured brain natriuretic peptide using Triage®, from Biosite, United States of America. We determined end-diastolic pressures of the right ventricle, and the peak ratio of right to left ventricular pressures, by cardiac catheterization and correlated them with concentrations of brain natriuretic peptide in the plasma. Results: The mean concentrations of brain natriuretic peptide were 87.7, with a range from 5 to 316, picograms per millilitre. Mean end-diastolic pressure in the right ventricle was 5.6, with a range from 2 to 10, millimetres of mercury, and the mean ratio of right to left ventricular pressure was 0.56, with a range from 0.24 to 1.03. There was a positive correlation between the concentrations of brain natriuretic peptide and the ratio of right to left ventricular pressure (r equal to 0.7844, p less than 0.0001) in all patients. These positive correlations remained when the children with tetralogy of Fallot, and those with atrial septal defects or anomalous pulmonary venous connection, were analysed as separate groups. We also found a weak correlation was shown between end-diastolic right ventricular pressure and concentrations of brain natriuretic peptide in the plasma (r equal to 0.5947, p equal to 0.0004). Conclusion: There is a significant correlation between right ventricular haemodynamic parameters and concentrations of brain natriuretic peptide in the plasma of children with right ventricular overload due to different types of congenital cardiac disease. The monitoring of brain natriuretic peptide may provide a non-invasive and safe quantitative follow up of the right ventricular pressure and volume overload in these patients.

Objectives: To identify predictive factors of complications occurring during paediatric interventional catheterisation. Background: Interventional paediatric catheterisation is still burdened by a substantial risk. Risk factors, however, have rarely been investigated. Methods: We analysed prospectively 1,022 interventional procedures performed over a period of 8 years, excluding 260 procedures for atrial septostomy. We considered several patient-related variables, specifically age, weight, and gender, type of procedure, times required for fluoroscopy and the overall procedure, technical challenge, and the severity of the clinical condition. We also analysed variables linked to the environment, specifically the date of the examination, whether the operator remained in training, the novelty of the material, any breakdown in the installation, and errors made by the operator. We classified complications as those without clinical consequence, those which proved lethal, those requiring cardiopulmonary resuscitation, elective or emergency surgery, hospitalisation in the intensive care unit, and those leading to recatheterisation. Results: Our average incidence of complications was 4.1 per cent, which did not change significantly during the period of study. Of the patients, 4 died, 7 needed urgent surgery, 5 elective surgery, 3 hospitalisation in intensive care unit, and 8 recatheterisation. Independent risk factors for complications were technical challenge, critical clinical condition, operator in training, operator error, and breakdown of the installation. Young age was not associated with a higher risk of complications. Patients in whom no cause for complication could be found, either related to their own features or the environment, had a risk of complication of 1.4 per cent (95 per cent confidence intervals from 0.7 to 2.5 per cent). Conclusions: Our data show that variables relating either to the patient or the environment of catheterisation are associated with an increased risk of procedural complications. Knowledge of the risk factors can improve the odds of paediatric interventional catheterisation.

Our experiments using in vivo labeling techniques in the chick embryo heart have demonstrated that the straight tube heart is constituted exclusively by the primordium of the trabeculated portion of the right and left ventricles. There are, therefore, no primitive cardiac cavities. Furthermore, in the stage of looping, two new ventricular components appear, namely, the inlet and the outlet. This new information on the normal development of the heart is important in order to understand both the anatomy of the normal heart and the development of congenital malformations. Due to the fact that the straight tube heart is constituted exclusively by the primordiums of the ventricular trabeculated portions the anatomic identification of the ventricles should be made on the basis of their apical trabeculated regions. The appearance of the components of the inlet and the outlet during the process of looping is the reason for the similarity in the congenital pathology of both regions, namely, a common arterial trunk and atrioventricular septal defect with common orifice. Because the trabeculated portions of the ventricles are the oldest developmental components, they form the basis on which malformations of the inlet or the outlet, or both, are superimposed.

Collaborative quality improvement and learning networks have amended healthcare quality and value across specialities. Motivated by these successes, the Pediatric Acute Care Cardiology Collaborative (PAC3) was founded in late 2014 with an emphasis on improving outcomes of paediatric cardiology patients within cardiac acute care units; acute care encompasses all hospital-based inpatient non-intensive care. PAC3 aims to deliver higher quality and greater value care by facilitating the sharing of ideas and building alignment among its member institutions. These aims are intentionally aligned with the work of other national clinical collaborations, registries, and parent advocacy organisations. The mission and early work of PAC3 is exemplified by the formal partnership with the Pediatric Cardiac Critical Care Consortium (PC4), as well as the creation of a clinical registry, which links with the PC4 registry to track practices and outcomes across the entire inpatient encounter from admission to discharge. Capturing the full inpatient experience allows detection of outcome differences related to variation in care delivered outside the cardiac ICU and development of benchmarks for cardiac acute care. We aspire to improve patient outcomes such as morbidity, hospital length of stay, and re-admission rates, while working to advance patient and family satisfaction. We will use quality improvement methodologies consistent with the Model for Improvement to achieve these aims. Membership currently includes 36 centres across North America, out of which 26 are also members of PC4. In this report, we describe the development of PAC3, including the philosophical, organisational, and infrastructural elements that will enable a paediatric acute care cardiology learning network.

Aim: Subclinical valvar insufficiency, or valvitis, has recently been identified using Doppler echocardiography in cases of acute rheumatic fever with isolated arthritis or chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is critical when determining the duration of antibiotic prophylaxis. We aimed, therefore, prospectively to investigate the association of silent valvitis in patients having rheumatic fever in the absence of clinical evidence of cardiac involvement, and to evaluate its prognosis. Methods and Results: Between November 1998 and September 1999, we identified 26 consecutive patients with silent valvitis in presence of rheumatic fever but in the absence of clinical signs of carditis. The patients, eight female and 18 male, were aged from 6 to 16 years, with a mean of 9.9± 2.7 years. Major findings were arthritis in 16, chorea in 7, and arthritis and erythema marginatum in 1 patient. Two cases had arthralgia with equivocal arthritic signs and Doppler echocardiographic findings of pathologic mitral regurgitation. Silent pathologic mitral regurgitation was found in 12 cases, and aortic regurgitation in 2 cases. All patients with arthritic findings were treated with acetylsalicylic acid with one exception, this patient receiving both prednisone and acetylsalicylic acid. No antiinflammatory treatment was given to patients with chorea. After a mean follow-up of 4.52 months, valvar regurgitation disappeared in 4 patients, including the one with migratory arthralgia and no other major criterions. All six patients with chorea and silent carditis still have mitral insufficiency. Conclusion: Acute rheumatic fever without clinical carditis is not a benign entity. Doppler echocardiographic findings of subclinical valvar insufficiency, therefore, should be considered as carditis when seeking to establish the diagnosis of acute rheumatic fever.

CHD is frequently associated with a genetic syndrome. These syndromes often present specific cardiovascular and non-cardiovascular co-morbidities that confer significant peri-operative risks affecting multiple organ systems. Although surgical outcomes have improved over time, these co-morbidities continue to contribute substantially to poor peri-operative mortality and morbidity outcomes. Peri-operative morbidity may have long-standing ramifications on neurodevelopment and overall health. Recognising the cardiovascular and non-cardiovascular risks associated with specific syndromic diagnoses will facilitate expectant management, early detection of clinical problems, and improved outcomes – for example, the development of syndrome-based protocols for peri-operative evaluation and prophylactic actions may improve outcomes for the more frequently encountered syndromes such as 22q11 deletion syndrome.

Background: Non-invasive measurement of left ventricular output has been shown to be a repeatable technique. Little is known about the repeatability using echocardiography in determining pulmonary arterial diameters or right ventricular output. Aims: To find the most repeatable point at which to measure pulmonary arterial diameter, and to compare the repeatability of determining right ventricular output with left ventricular output. Methods: We assessed the Intra-observer and inter-observer repeatability for measuring the diameter of the pulmonary trunk in 24 term and 26 preterm infants, respectively. Interobserver repeatability was assessed for the diameters of the pulmonary trunk and aorta, for stroke distance, and for left and right ventricular output. Results: The coefficients of variation for intra-observer repeatability were 4%, 7.5% and 9% respectively for measurements of the pulmonary valve, the pulmonary trunk, and the right ventricular outflow tract. There were significant differences between observers for measurement of the pulmonary trunk (p< 0.001) and right ventricular outflow tract (p= 0.011) but not for the pulmonary valve measured in either its long (p= 0.22) or short axis (p= 0.22). Significant differences between observers were also found for the pulmonary stroke distance measured in the long axis (p= 0.004) and aortic diameter at end-diastole (p< 0.001). The other parameters did not differ significantly and were used to calculate right and left ventricular output, respectively. Mean left ventricular output was 241 mls/kg/min, with mean differences between observers of 0.6 mls/kg/min (95% confidence interval (CI): −39.2 to 40.3 mls/kg/min). Mean right ventricular output was 255 mls/kg/min, with mean differences of 0.3 mls/kg/min (95% CI: −24.1 to 23.4 mls/kg/min). Conclusion: Measuring the diameter of the pulmonary trunk at the base of the valvar hinge points was most repeatable. Repeatability of right ventricular output was similar to that of left, with absolute values similar to those published by other workers.

Paediatric myocarditis remains challenging from the perspectives of diagnosis and management. Multiple aetiologies exist and the majority of cases appear to be related to viral illnesses. Enteroviruses are believed to be the most common cause, although cases related to adenovirus may be more frequent than suspected. The clinical presentation is extremely varied, ranging from asymptomatic to sudden unexpected death. A high index of suspicion is crucial. There is emerging evidence to support investigations such as serum N-terminal B-type natriuretic peptide levels, as well as cardiac magnetic resonance imaging as adjuncts to the clinical diagnosis. In the future, these may reduce the necessity for invasive methods, such as endomyocardial biopsy, which remain the gold standard. Management generally includes supportive care, consisting of cardiac failure medical management, with the potential for mechanical support and cardiac transplantation. Treatments aimed at immunosuppression remain controversial. The paediatric literature is extremely limited with no conclusive evidence to support or refute these strategies. This article summarises the current literature regarding aetiology, clinical presentation, diagnosis, and management of myocarditis in paediatric patients.

We evaluated the efficiency of non-invasive magnetic resonance coronary angiography in detecting coronary arterial lesions in 106 patients, aged from 4 months to 37 years, with a median of 13 years, with Kawasaki disease. Non-contrast enhanced, free-breathing magnetic resonance coronary angiographic studies using both the steady-state free precession technique, namely bright blood imaging, and navigator-echo proton density weighted black blood imaging, so-called black blood imaging, were performed in all the patients. Conventional X-ray coronary angiography was performed in 70 patients with coronary arterial lesions.

We observed 97 aneurysms, 17 dilatations, 17 occlusions, 18 localized stenoses and 10 recanalized vessels, and we clarified their unique pattern of images on magnetic resonance coronary angiography. The differences in size of the aneurysms as seen on X-ray coronary angiography and bright blood imaging was mean 0.0, and the 95% confidence interval was from −1.4 to 1.5 on the Bland-Altman plots. With bright blood imaging, the sensitivity of occlusion and localized stenosis based on X-ray angiography was 94.2% and 97.2%, specificity was 99.5% and 97.2%, and negative-predictive value was 99.5% and 97.2%, respectively. Black blood imaging provided remarkable visualization of the thickened intima of aneurysms, and/or thrombus, in 38 lesions. We conclude that magnetic resonance coronary angiography can visualize all types of lesions due to Kawasaki disease in patients of all ages, and that it is useful to reduce the number of times X-ray angiography needs to be performed in patients with Kawasaki disease.