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.

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.

Over a 25-year period, we encountered 12 patients who died suddenly with coronary arterial lesions due to Kawasaki disease. We report their clinical course, and analyze the happenings of their deaths. Of the 12 patients, 10 were dead on arrival at hospital. Their age at death ranged from 13 months to 27 years, with a median of 16 years, and the interval from the onset of Kawasaki disease to death ranged from 2 months to 24 years. In 4 patients, death was found to be due to myocardial infarction, while in the remaining 8, it could not be determined. In 7 patients, coronary angiograms obtained less than 4 months after the acute onset of Kawasaki disease showed lesions bilaterally, most being giant aneurysms. Myocardial infarction had occurred in 6 patients prior to their death. In 1 patient of the late 1970s, who collapsed after running, cardiac sequels had not been suspected prior to autopsy. During the 1980s, 3 infants with bilateral giant aneurysms died within a year of the initial onset of Kawasaki disease, with acute myocardial infarction being the cause in 2 of them. In the late 1990s, and the 2000s, 5 patients died suddenly with left ventricular dysfunction, their ejection fractions being less than 40 percent more than 20 years after the initial onset of Kawasaki disease. Prior to their sudden deaths, they had had no cardiac events for many years, but had suffered previous myocardial infarctions. Multifocal premature ventricular contractions, and non-sustained ventricular tachycardia, are probable risk factors in such patients. Careful follow-up, checking for ventricular arrhythmia, is needed to prevent sudden death in patients suffering left ventricular dysfunction in the setting of Kawasaki disease.

The provision of outreach services by paediatric cardiac centres enhances the choice for both parents and professionals. We have conducted a survey to investigate the processes for information and communication at times of interface between specialist and local services for cardiac disease. Based on the responses, we suggest that distance from, and level of contact with, the specialist centre may influence satisfaction and the perceived usefulness of more information. We suggest strategies to increase contact, and make more effective targeting of resources.

Objectives: To determine whether the present system of reimbursement, based on diagnosis-related groups and regular financial budgeting, covers the costs incurred during hospitalisation of 7 children undergoing the three stages of the Norwood sequence for surgical treatment of hypoplastic left heart syndrome. Methods: Between January and September 2003, 7 patients underwent initial surgical palliation with the Norwood procedure at the Rikshospitalet University Hospital. A prospective methodology was developed by our group to measure the costs associated with each individual patient. The patients were closely observed, and the relevant data was collected during their stay in hospital. The stay was divided into four different periods of requirements of resources, defined as heavy intensive care, light intensive care, intermediate care, and ordinary care. At each stage, we recorded the number of staff involved and the duration of surgery and other major procedures, as well as the cost of pharmaceuticals and other consumables. Based on these data, we calculated the cost for each patient. These costs were compared with the corresponding revenue received by the hospital for each of the patients. Results: We found the total mean cost for the three stages of the Norwood sequence was 138,934 American dollars, while the corresponding revenue received by the hospital was 43,735 American dollars. During this period, one patient died during the first stage of the Norwood sequence. Conclusions: Our study shows that steps involved in the Norwood sequence are low-volume but high-cost procedures. The reimbursement received by our hospital for the procedures was less than one-third of the recorded costs.

Autonomic nervous control of the heart can be studied by analysing variability in heart rate. Although earlier studies have shown reduced variability in patients with the Fontan circulation, we are not aware of any previous study examining longitudinal changes in such children. We have examined 13 patients who had undergone total cavopulmonary connection, and 37 healthy controls matched for age and gender. The examinations included complete echocardiography, and 24-hour ambulatory electrocardiogram for analysis of the parameters for variability in heart rate. After the Fontan procedure, three follow-up examinations were performed at a mean of 4.4 years, 5.6 and 7.2 years. Reduced variability was found in those with the Fontan circulation. A significant difference was found between patients and their controls with respect to high-frequency power at the second, p equal to 0.05, and third, p equal to 0.03, examination. The ratio of low-to-high-frequency components progressively increased in those with the Fontan circulation, a phenomenon that led to a significant difference, p equal to 0.03, at the third examination. Our study shows that, in patients with the Fontan circulation, routine ambulatory electrocardiographic monitoring including analysis of variability in heart rate, detects over time a progressive sympatovagal imbalance.

Endothelial dysfunction has been reported in hypoxaemic patients with the Eisenmenger syndrome, but a direct correlation between levels of endothelial markers and the severity of hypoxaemia has not been explored. With this in mind, we compared the levels in the plasma of tissue-type plasminogen activator, thrombomodulin, and von Willebrand factor in 25 patients with the Eisenmenger syndrome. They had a median age of 31 years, and were divided into 2 groups according to their recent clinical history. Thus, 18 patients were stable, being in functional class II or III, seen as outpatients, and having peripheral saturations of oxygen of 89 plus or minus 5 percent. In contrast, 7 patients were unstable, showing episodes of symptoms placing them in functional class IV, requiring care in hospital, and manifesting saturations of oxygen of 77 plus or minus 5 percent. We were able to follow 12 patients, 8 who were stable and 4 unstable, for 24 months. At baseline, levels of von Willebrand factor were higher in the unstable patients when compared to those who were stable, at 142 plus or minus 29 and 110 plus or minus 25 units per decilitre, respectively (p equal to 0.013). This correlated positively with oxygen desaturation (p less than 0.020). The structural abnormalities also correlated positively with the magnitude of hypoxaemia (p less than 0.020). Levels remained higher in the unstable patients throughout the period of follow-up (p equal to 0.006). Tissue-type plasminogen activator was also increased, at 14.3 plus or minus 8.4 versus 6.5 plus or minus 2.7 nanograms per millilitre in controls (p less than 0.001), whereas thrombomodulin was decreased, with values of 14.4 versus 34.6 nanograms per millilitre in controls (p for median values of less than 0.001). There was no correlation with saturations of oxygen. We conclude that measurement of von Willebrand factor, as compared with tissue-type plasminogen activator and thrombomodulin, will prove a better marker of endothelial response to hypoxaemia in patients with the Eisenmenger syndrome.

Patients with acute rheumatic fever sometimes present with atypical signs and symptoms. In these circumstances, the Jones criterions may not be sufficient to make a clinical diagnosis. We describe here two patients with unusual presentations, highlighting that, both in regions where the disease is endemic, or where it is seen only sporadically, physicians should be more alert and careful in making the diagnosis.

I-cell disease is a rare, hereditary, and fatal disease, which is often complicated with disease of the cardiac valves. We have experienced a case of severe mitral valvar insufficiency in a 9-month-old male infant with I-cell disease. Mitral valvoplasty was difficult, because the leaflets were remarkably hypertrophic, and the tendinous cords were elongated and adherent. Despite these difficulties, surgery proved effective in the treatment of the congestive heart failure, allowing for further treatment of the I-cell disease.

We describe a 9-year-old girl who was noted to have eosinophilic myocarditis in her native heart after it was explanted during cardiac transplantation. On the basis of absence of evidence for primary or secondary eosinophilia, we suggest that the prolonged use of dobutamine prior to transplantation might have induced the eosinophilic myocarditis.

To date, almost all patients reported with hypoplasia of the left heart have had usual atrial arrangement, with a small proportion known to have left or right isomerism. As far as I am aware, however, only one patient has previously been described with mirror-imaged arrangement of the heart and organs. In this report, I describe the second case.

Aortic coarctation is occasionally detected only in adulthood, usually in the context of systemic hypertension. It is frequently associated with other malformations, but the presence of severe disease of the aortic or mitral valves is rare. Such associated lesions, nonetheless, have important implications regarding the type and timing of therapeutic interventions. We describe an adult patient with severe aortic coarctation, aortic valvar regurgitation, and impaired left ventricular systolic function. We treated the aortic coarctation first by means of percutaneous dilation and implantation of a stent. Four days later, we proceeded to treat the aortic regurgitation surgically, using the Bentall procedure. Our experience documents a safe and efficient therapeutic approach to this association of lesions.

We report a fatal dissection of the descending aorta as a complication after a two-staged implantation of a stent to relieve aortic coarctation in a young female with Turner's syndrome. Implantation of the stent, with dilation up to 70 percent, and half a year later re-dilation to 100 percent, was without any complication. A week after the re-dilation, however, the patient suffered acute dissection of the descending aorta. We initially stabilized the situation, but some days later her condition deteriorated, and she died. We discuss the various options for treating coarctation and re-coarctation. As far as we know, this is the first description of implantation of a stent in the setting of coarctation in Turner's syndrome, albeit with an unfortunate conclusion.

As far as we are aware, right-sided bacterial endocarditis has not previously been described as presenting with systemic illness and testicular swelling. We report a teenager who presented with this unusual combination as a consequence of right-sided endocarditis. He presented with high fever, with chills and rigor, along with painful enlargement of the left testicle, a productive cough with progressive breathlessness, and joint pains. His blood culture was positive for Staphylococcus aureus, and a computerised tomographic scan of the chest revealed multiple pulmonary emboluses. Ultrasound of the testicles showed features of inflammation, and an echocardiogram revealed a vegetation on the tricuspid valve.

We describe a 6-year-old boy with a right-sided heart part of the Kartagener syndrome, complicated by presence of a perimembranous ventricular septal defect. The defect was closed interventionally using the Amplatzer™ asymmetrical occluder for ventricular septal defects. The procedure was event-free. Non-operative closure of a ventricular septal defect in this patient demonstrated no other difficulty than mirror-imaged thinking, thus giving evidence that such treatment also can be offered to patients with positional anomalies.