Pulmonary vascular disease is a risk factor for the surgical management of common arterial trunk. Surgical repair, therefore, is usually performed in early infancy, before irreversible changes can occur in the epulmonary vasculature. Because of this, there has been an increasing tendency to dispense with cardiac catheterisation as a means of assessing pulmonary vascular disease. Cardiac catheterisation, nonetheless, is still performed when there is a risk of pulmonary vascular disease, such as in older children. There are no clear guidelines, however, as to who should be catheterised. We have developed a simple screening test to help make this decision.

Traditionally, medical professionals have entrusted the parents of children with chronic illness with the task of imparting knowledge about the illness to the children. This practice assumes that parents understand the illness, and that they pass on their knowledge in a manner appropriate for the individual child. The aim of our study was to assess the knowledge that parents of children with a cardiac defect have about the malformation in the heart of their child. We sent a modified version of the Leuven questionnaire to 350 families. The questionnaire was filled in and sent back to our centre by 148 families. Parents showed a good knowledge of the name and anatomical characteristics of the cardiac disease suffered by their child. Parents with a child taking drugs were not very knowledgeable about the regime, side effects, and interaction with other drugs or food. Only one-quarter of the parents knew the definition of endocarditis, although almost two-fifths were aware that unexplained fever for more than 5 days was the most typical symptom. Less than half of the parents knew that endocarditis could recur. About two-fifths of parents knew the real possibility of their child being involved in competitive sports; but almost half of parents were unable to answer this question. The poor knowledge about particular aspects of the disease, treatment, and preventive measures revealed by our parents may have major consequences. The results of our study are relevant to general daily clinical practice.

Aim: To identify patients with visceral heterotaxy who are at risk from fulminant sepsis. Methods: We studied 38 patients, 37 having undergone abdominal ultrasound, all 38 having examination of blood films to establish presence of Howell-Jolly bodies, and all 38 documented to have had pneumococcal vaccination and prophylaxis with penicillin. We checked whether the parents were aware of the splenic state of their child, and when possible, we compared current results of blood films with those obtained postnatally. Results: Two of the 17 patients with multiple spleens, all 11 without a detectable spleen, and 1 of 9 patients with a normal spleen, showed Howell-Jolly bodies in their blood films. In 5 of 23 patients with serial blood films, Howell-Jolly bodies had not been seen postnatally, but could now be detected in current blood films. Of these patients, 2 had multiple spleens, 1 did not have a spleen, and 1 had a solitary spleen of normal size. In the other patient, ultrasound could not be performed. Only one of these patients was receiving penicillin prophylactically, and had received pneumococcal vaccination. Of the 15 patients in whom Howell-Jolly bodies were present in the blood, only 8 parents knew about the potential risk for infection. Another 7 parents were sure that their child was taking penicillin regularly, and had received pneumococcal vaccination. Conclusions: Howell-Jolly bodies can be found in the blood of patients with visceral heterotaxy independent of the anatomical state of the spleen. As Howell-Jolly bodies can be encountered in the blood of such patients with increasing age, those with multiple and solitary spleens should be monitored regularly to identify those at risk. Parental knowledge of the splenic state, and compliance for prophylaxis using penicillin, and pneumococcal vaccination, were unsatisfactory in our cohort.

The previous reviews in this section of our Supplement1,2 have summarized the anatomic components of the ventriculo-arterial junctions, and then assessed the echocardiographic approach to the ventriculo-arterial junction or junctions as seen in the morphologically right ventricle. In this complementary review, we discuss the echocardiographic assessment of the comparable components found in the morphologically left ventricle, specifically the outflow tract and the arterial root. We will address the echocardiographic anatomy of the aortic valvar complex, and we will review the causes of congenital arterial valvar stenosis, using the aortic valve as our example. We will also review the various lesions that, in the outflow of the morphologically left ventricle, can produce subvalvar and supravalvar stenosis. We will then consider the salient features of the left ventricular outflow tract in patients with discordant ventriculo-arterial connections, and double outlet ventricles. To conclude the review, we will briefly address some rarer anomalies that involve the left ventricular outflow tract, showing how the transesophageal echocardiogram is used to assist the surgeon preparing for repair. The essence of the approach will be to consider the malformations as seen at valvar, subvalvar, or supravalvar levels,1 but we should not lose sight of the fact that aortic coarctation or interruption, hypoplasia of the left heart, and malformations of the mitral valve are all part of the spectrum of lesions associated with obstruction to the left ventricular outflow tract. These additional malformations, however, are beyond the scope of this review.

Increasing numbers of children who have undergone intracardiac repair of tetralogy of Fallot have survived to adulthood, but often have residual haemodynamic and/or electrical abnormalities. We report the unusual observation of 4 instances of cerebrovascular accidents in young adults subsequent to surgical repair of tetralogy of Fallot.

Objective: Primary implantation of stents is an accepted technique for treating aortic recoarctation, albeit that the effects of stenting on pressure profiles, carotid mechanical properties, intimal medial thickness, and reflection of the pulse wave have not been systematically investigated. Methods: Over the period from 1 January, 1999, to 31 December, 2002, we implanted stents to relieve aortic recoarctation in 15 patients, with a median age of 17 years, and a range from 7 to 29 years, with a median weight of 56 kilograms, ranging from 20 to 96 kilograms. Indications were a gradient of 20 millimetres of mercury or more measured in all, systemic hypertension at rest in 8, and systemic hypertension at exercise in all. Of the patients, 5 were receiving anti-hypertensive treatment. Before implantation of the stents, and after a mean follow-up of 22 months, all patients underwent an exercise test, vascular echography, and examination of the common carotid artery so as to determine its cross sectional compliance and distensibility, and the augmentation index. Results: The stents were implanted successfully in all patients. The mean gradient was reduced from 27 to 4 millimetres of mercury (p < 0.001). Systolic blood pressure at rest diminished from 140 to 131 millimetres of mercury (p = 0.04), while hypertension at rest regressed in 4 patients. Systolic blood pressure at exercise diminished from 245 to 222 millimetres of mercury (p = 0.018), and hypertension at exercise regressed in 1 patient. Anti-hypertensive treatment is still required for 4 patients. A correlation was found between systolic blood pressure at rest and initial peak-to-peak gradient (r = 0.8), and between initial gradient and percentage reduction of systolic blood pressure at rest at follow-up (r = −0.73). Compliance and distensibility of the common carotid artery were not significantly modified, albeit that the intimal medial thickness diminished from 0.64 to 0.57 millimetres (p = 0.04), and the augmentation index decreased from 5 to −1 (p = 0.012). Conclusions: Primary implantation of stents is effective in mid-term repair of aortic recoarctation. Although there is an improvement in systemic hypertension, the tensional profile and vascular sonography are not normalized. At long term follow-up, the suppression of an early reflection site of the pulse wave could decrease the wall stress of the great elastic vessels, reducing the thickness of the arterial walls.

The concept that the adult heart is a terminally differentiated organ has been generally accepted for many years. As a post-mitotic organ, the heart has been considered to be characterized by a predetermined number of parenchymal cells. This number is held to be defined at birth, and is preserved until death of the organ and/or the organism. According to this concept, the increase of myocardial mass, or in other words, myocardial hypertrophy, is interpreted on the basis of growth of single terminally differentiated myocytes, able to produce contractile elements and organelles, but not capable of division. Ventricular remodelling has been envisaged, therefore, as a balance between the phenomenon leading to either increase in cardiac mass, in other words hypertrophy, or loss of myocytes, as occurring in apoptosis or necrosis.

Background: Post-operative junctional ectopic tachycardia is a transient, but potentially life threatening, rapid automatic tachyarrhythmia that requires urgent and adequate treatment. In our study, we review retrospectively the use and efficacy of amiodarone for this arrhythmia over an 8-year period in our institution. Methods and patients: Retrospective review revealed 15 patients who were administered amiodarone for post-operative junctional ectopic tachycardia during the period. The median age was 2.6 months, with a range from 8 days to 8.1 months. The median weight was 4.6 kilograms, with a range from 2.6 to 8.2 kilograms. Results: The median heart rate at diagnosis of the tachycardia was 192 beats per minute, and the range was 182 to 229 beats per minute. The biochemistry was essentially normal. The median length of time until the tachycardia was controlled was 4.5 hours, and the range was from 1 to 19.5 hours, with 13 of the 15 patients controlled within 12 hours. The median dose of amiodarone received by this time was 5.9 milligrams per kilogram, with a range from 1.0 to 25.0 milligrams per kilogram. Hypotension or bradycardia within 4 hours of commencing amiodarone were noted in 2 patients. Conclusion: Experience in our institution, and a review of the literature, suggests that the most rapid control of post-operative junctional ectopic tachycardia will be obtained by a bolus of amiodarone followed by an intravenous infusion. Intravenous amiodarone is generally safe, with few side effects. Reported life threatening arrhythmias, however, suggest that intravenous amiodarone should be restricted to a setting where invasive monitoring and external cardiac pacing are available.

We measured resting and exercise haemodynamics, as well as 24-hour ambulatory blood pressure, so as to study the influence on development of hypertension in children after repair of coarctation by either construction of a subclavian flap or end-to-end anastamosis. The patients in both groups were studied a mean time of 13 years after surgery. Thus, we divided 43 children who had undergone surgical repair of coarctation, and who were not on antihypertensive therapy, into a group of 22 patients who had undergone subclavian flap repair, with a mean age of 14 plus or minus 2.6 years, and another group of 21 patients undergoing end-to-end anastomosis, with a mean age of 13.5 plus or minus 3.9 years. We examined blood pressure at rest and during exercise, along with the measurement of cardiac output using impedance cardiography, and during 24-hour ambulatory monitoring. We recorded systolic and diastolic blood pressures, pulse pressure, cardiac output and total peripheral vascular resistance at rest and at peak exercise. During ambulatory monitoring, we measured mean pressures over 24 hours, in daytime and nighttime, 24-hour pulse pressure, and 24-hour mean arterial pressure. Student's t test was used to judge significance, accepting this when p was less than 0.05. The group repaired using the subclavian flap showed significantly disadvantageous differences for diastolic blood pressure at rest, systolic blood pressure at peak exercise and for 24-hour systolic and diastolic blood pressure, 24-hour mean arterial pressure, and daytime and nighttime systolic blood pressure during ambulatory monitoring. Our findings suggest that, after repair using the subclavian flap in comparison to end-to-end anastomosis, patients show a higher incidence of late hypertension, both during exercise and ambulatory monitoring. The data indicate different residual aortic stiffnesses, these being lower after end-to-end anastomosis, which may be due to the greater resection of the abnormal aortic tissue when coarctation is repaired using the latter technique.

Aims: Firstly to assess maternal perceptions of the impact of congenital or acquired cardiac disease on the child, parents, and siblings, and secondly to determine whether there were differences between different diagnostic groups, or between those with and without other health problems, with a view to informing the development of a cardiac liaison nursing service for children. Methods: A postal survey of 447 families of children with congenital or acquired cardiac disease. Results: Completed questionnaires were received from 209 (46.8 percent) families. The cardiac lesion was perceived to have a negative impact on many areas of family life for about one fifth of the sample, particularly in those families where the child was perceived to be more ill. Family relationships, however, were affected in a very different way, with 43 percent reporting that family members had become closer, and only 8 percent that they had been “pulled apart” by the condition of their child. There were a number of differences in the perceived impact of the cardiac malformation on school and family life between children with different diagnoses, with this being particularly evident for families of the patients who had undergone transplantation. When the sample was divided according to the presence or absence of other problems with health, however, many of these differences between the diagnostic groups disappeared. Conclusions: Irrespective of the severity of the disease, the presence of a cardiac malformation has an impact on everyday life for a significant number of children and families, particularly if associated with other problems with health. Implications for targeting resources to reduce morbidity in these children and families are discussed.

Aims: To determine the number of infants in the Mersey and North West regions with congenital cardiac disease for whom palivizumab may be appropriate, and to examine the potential impact of introducing prophylaxis with palivizumab on these patients and their economic management. Methods: We identified those infants deemed to be at high risk, matching the population recently studied by the Cardiac Synagis Group, from the database of the cardiology department of the hospital. The number of patients under the care of the paediatric cardiologists admitted to Alder Hey Hospital with respiratory syncytial viral bronchiolitis over the last three seasons was identified from hospital coding records, and the database of the cardiology department. Results: There are 131 patients at high risk each year. Of these, over the last three “bronchiolitis seasons”, 39 infants have been admitted to the hospital with bronchiolitis due to the respiratory syncytial virus. This represents a hospitalisation rate of 10 per cent, as was seen in the study of the Cardiac Synagis Group. Using a monthly dose of 15 milligrams per kilogram for five doses, the cost per patient is 2,650 pounds sterling for the season. To treat the 131 patients seen at Alder Hey, therefore, would cost 346,800 pounds each year. Applying the reductions in hospitalisation identified in the study by the Cardiac Synagis Group to our population would produce an expected reduction in patients hospitalised from 13 to 7 per year, reducing the total length of stay in our hospital wards from 169 to 76 days, and in the paediatric intensive care unit from 93 to 21 days. This amounts to a potential saving of 190,800 pounds per year. Reducing transfers to more distant paediatric intensive care units for referrals refused because of lack of beds could save an additional 50,000 pounds. Discussion: We estimate the net cost of introducing palivizumab for this population to be 106,000 pounds per year. There would, of course, be additional costs involved in setting up this service, as well as additional savings and benefits. This cost is comparable with other new biologic therapies now routinely used in the United Kingdom, such as etanercept for juvenile arthritis. There are, currently, no other obvious therapies that have the potential to reduce admissions to hospital and intensive care during the winter months, when beds are at their most scarce.

Many complex cardiac defects produce a functionally single ventricle, in which there is mixing of the systemic and pulmonary circulations. The output from the functionally single ventricle is divided between the two circulations: the proportion going to the systemic and pulmonary vascular beds being determined by the relative resistance to flows within the respective circulations.

In this study, we investigated some of the prothrombothic mutations and polymorphisms in 15 children with congenital cardiac malformations who developed severe thrombosis in the perioperative period following surgical repair. The mutations and polymorphisms included in the study were Factor V Leiden, prothrombin G20210A, methylentetrahydrofolate reductase C677T, endothelial nitric oxide synthase intron 4 VNTR, alpha-fibrinogen Thr312Ala, Factor XIII Val34Leu, and insertion or deletion of angiotensin 1 converting enzyme. Compared to the healthy Turkish subjects, our patients had a similar rate of mutation of Factor V Leiden, Factor XIII Val34Leu, and endothelial nitric oxide synthase a/b polymorphisms, but higher frequency of the prothrombotic angiotensin 1 converting enzyme deletion/deletion genotype, and lower frequency of the antithrombotic alpha fibrinogen Thr/Thr genotype. None of the patients exhibited mutations involving prothrombin G20210A or methylentetrahydrofolate reductase C677T. The results of our study suggest that, in addition to prothrombotic mutations such as Factor V Leiden, single-nucleotide polymorphisms should be considered in all children with congenital cardiac malformations who develop thrombosis.

Malformations of the heart are the most common of all serious lesions that are present at birth, with an incidence of 4 to 8 cases per 1,000 live births.1 If needed, corrective surgery is usually the optimal treatment for these anomalies, but perioperative morbidity and mortality still remain high due to several factors. Arterial or venous thrombosis, or both varieties of thrombosis, is among these factors. Prior to surgery, the most frequent time at which these children develop thrombosis is during cardiac catheterization. Postoperative thrombosis in this group of patients is a more complex disorder, which can affect both small and large vessels, and is associated with a high morbidity and mortality.

Recent studies indicate that both point mutations and single-nucleotide polymorphisms of genes that encode proteins involved in the coagulative and anticoagulative cascades are important risk factors for development of thrombosis. Patients with these risk factors are most likely to develop thrombosis when triggering elements, such as placement of catheters, prolonged immobilization, or surgery, are also present. In this study, we investigated some of the above-mentioned mutations and polymorphisms in children who developed thrombosis in the perioperative period after correction of congenital cardiac malformations.