In this issue of the Journal, we publish three important papers concerned with rheumatic fever. So as to put these contributions in their world-wide perspective, I asked Cleonice Mota, a member of our Editorial Board, if she would prepare the “From the Editor” section for this issue. It is our pleasure to publish her thoughtful introduction, and we thank her for her continuing efforts on our behalf.

Robert H. Anderson

Carditis is the only manifestation of acute rheumatic fever that leads to permanent disability. Hence, its diagnosis is of paramount importance. Recently, it has been reported that Doppler echocardiography has disclosed subclinical valvar regurgitation in some patients with acute rheumatic fever manifested as isolated arthritis or pure chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is not clear. We aimed, therefore, prospectively to investigate the potential to diagnose patients with subclinical carditis. We examined 40 patients, aged from 7 to 16 years, with Doppler evidence of mitral and aortic regurgitation, but in the absence of any pathologic murmur. The major findings satisfying the Jones criterions were arthritis in 29 patients, chorea in 10 patients, and arthritis and erythema marginatum in one patient. Of the patients, 33 had mitral regurgitation, 6 patients had combined mitral and aortic regurgitation, and one patient had aortic regurgitation. The patients were followed over a mean period of 18.1 ± 13.9 months, the valvar regurgitation disappearing in 23 (57.5%). No significant differences were observed in the resolution of the valvitis between those treated with acetylsalicylic acid, steroids, or those receiving no treatment. It is noteworthy, nonetheless, that patients treated with steroids were the fastest to recover from valvitis (p < 0.05).

Based on our study, we suggest that subclinical valvitis demonstrated by echocardiography should now be accepted as adequate evidence for the diagnosis of carditis, and become a major diagnostic criterion for acute rheumatic fever. When managing this group of patients with subclinical disease, treatment with steroids seems to have a role in promoting early resolution of the valvitis.

Recent technical improvements in cross-sectional echocardiography have made it possible to detect even mild organic regurgitation of the mitral and aortic valves in patients with acute rheumatic fever. To determine the prevalence and prognosis of subclinical valvitis, we have analyzed 104 patients with acute rheumatic fever referred to our institution. Of 53 patients who had no murmur, 22 of them with polyarthritis, 29 with chorea, and 2 with polyarthritis and chorea, 23 (43.4%) had subclinical valvitis. Isolated mitral regurgitation was the most common valvar lesion, seen in 82.6% of the patients. Isolated aortic regurgitation was detected in 4.4% of the cases, and combined mitral and aortic regurgitation in the remaining 13%. During follow-up, the degree of mitral regurgitation improved in 59.1%, decreased in 18.2%, and increased or remained unchanged in 22.7% according to the length of colour jet. According to criterions of velocity, mitral regurgitation improved in 86.4% of the patients, and increased or unchanged in the remaining 13.6%. Mitral regurgitation disappeared completely in 6 of the patients (27.3%) as judged according to both the length of colour jet and the velocity of regurgitation. Aortic regurgitation improved in all the patients with this problem, disappearing completely in two of the four.

Based on this experience, we suggest that not only the disappearance of regurgitation, but also improvements in the echocardiographic diagnostic criterions of regurgitation, such as the length of the colour jet less than 1 cm, or velocity less than 2.5 m/s, or indicative of regurgitation that is either intermittent or of short duration, should also be considered as criterions indicating improvement in valvar regurgitation in patients with subclinical rheumatic valvitis.

We carried out a retrospective case control analysis to evaluate the outcome, and the need for treatment, of problems with atrioventricular conduction occurring during an acute attack of rheumatic fever, assessing the occurrence of second and third atrioventricular block versus first degree block.

We reviewed and analysed the clinical, electrocardiographic and echocardiographic records of all children diagnosed in a single institute as having acute rheumatic fever during a period of seven consecutive years.

During the period from October, 1994, through October, 2001, 65 children meeting the modified Jones criterions for acute rheumatic fever were hospitalized in the Soroka University Medical Center, Israel. First-degree atrioventricular block was identified in 72.3% of the children, and resolved with no specific treatment other than non-steroidal anti-inflammatory medications. Second-degree atrioventricular block of Mobitz type I, was observed in one child (1.5%), which progressed from first-degree block, and subsequently resolved. Complete atrioventricuar block was found in 3 children (4.6%), one progressing from Mobitz type I second-degree block, and two being seen as the first presentation. Of the three children with complete atrioventricular block, one patient was not treated, the second was treated with aspirin, and the final one with combined aspirin and steroids. The disturbances of conduction resolved in all three.

We conclude that advanced atrioventricular block is rare during acute rheumatic fever. If occurring, block appears to be temporary, and resolves with conventional anti-inflammatory treatment. Specific treatment, such as insertion of a temporary pacemaker, should be considered only when syncope or clinical symptoms persist.

Background: We reviewed our 12-year experience with staged reconstruction for hypoplasia of the left heart, examining the results of each surgical step and the impact of the year of the Norwood operation on survival. We compared survival of patients with hypoplasia of the left heart subsequent to completion of the Fontan circulation to survival of patients with a dominant left ventricle undergoing a Fontan procedure. Patients: Between 1989 and 2001, we performed a first stage procedure in 89 patients. Their median age was 9 days, with a range from 2 to 140 days, and the median weight was 3.4 kg, with a range from 2.4 to 5.4 kg. Results: Survival at 1, 4, and 10 years was 55%, 49%, and 49%, respectively. We experienced 23 early deaths (26%), and 12 deaths between the stages of the Norwood cascade. Of our patients, 42 underwent the second stage, and 30 the third stage. Prior to the first stage, symptoms of necrotising enterocolitis, and of obstructed pulmonary venous return, influenced survival significantly. The latter was eliminated as risk factor when surgery was performed within the first week of life. During the later part of our experience, survival at the first stage operation improved significantly, with survival at 3 years increasing from 42% to 75% for the patients at standard-risk (p = 0.017), and from 17% to 42% for those deemed to be at high-risk (p = 0.1). No deaths occurred in 23 patients older than 3 years of age, all of whom had proceeded through the third stage. After completion of the Fontan circulation, the survival of the patients with hypoplasia of the left heart at 4 years was comparable to the survival of patients undergoing the Fontan procedure with a dominant left ventricle (88% versus 90%, p = 0.8). Conclusions: Early and intermediate survival has improved significantly over the period of 12 years. Late death has been uncommon, and none of our patients are listed for cardiac transplantation.

We implanted either large or medium Palmaz stents, or a Palmaz Corinthian stent, in various stenotic vessels, such as the pulmonary arteries, pulmonary veins, aorta, or superior caval vein. Using angiograms, we measured the diameter of the stenotic vessel before or after the implantation, the minimal diameter of the lumen, the minimal diameter of the largest fully expanded balloon used to expand the stent, and the diameter immediately after withdrawal of the balloon.

The minimal diameter of the fully expanded balloon, and the minimal diameter of the lumen subsequent to expansion, were 8.2 ± 2.4, and 7.7 ± 2.3 mm, giving an absolute recoil of 0.5 ± 0.4 mm, and a proportional recoil of 7 ± 4%. There was no significant difference in either the absolute or proportional recoil for any of the stents, or for any of the different stenotic vessels. The proportional recoil correlated linearly with the minimal diameter of the lumen prior to the procedure, and with the ratio of the stenosis to the balloon, while the diameter of the stenotic vessels, the minimal diameter of the largest fully expanded balloon, the proportional stenosis prior to the procedure, and the ratio of the balloon to the diameter of the stenotic vessel, had no significant correlation with proportional recoil. The proportional recoil exceeded more than one-tenth when the minimal diameter of the lumen prior to the dilation was less than 3 mm, or the ratio of the balloon to the stenosis was greater than 3.0.

An absolute recoil of around 1 mm is common when a large or medium Palmaz, or a Palmaz Corinthian stent, is implanted in great vessels. Balloons with a diameter of approximately one-tenth greater than that of the adjacent vessel may be needed if the minimal diameter of the lumen is small prior to the procedure.

Background: To determine the rate of progression of dilation of the aortic root in adults with a bicuspid aortic valve. Methods: We reviewed retrospectively the transthoracic echocardiograms of 50 adults with a bicuspid aortic valve. Each patient had had at least two examinations made 12 months apart. Measurements were taken at four levels: at the basal attachment of the leaflets of the valve within the left ventricular outflow tract, at the widest point of the sinuses of Valsalva, at the sinutubular junction, and in the ascending aorta 1 cm beyond the sinutubular junction. Results: Progressive dilation occurred at all levels, ranging from 0.3 mm/yr at the basal attachment within the left ventricular outflow tract to 1.0 mm/yr, 1 cm beyond sinutubular junction. These rates of dilation were greater than the reported rate of 0.8 mm per decade in the normal population. The rate of dilation found in the ascending aorta 1 cm beyond the sinutubular junction was significantly greater than at the other sites (p = 0.005). The 21 patients with baseline measurements greater than 34 mm had a significantly higher rate of progression (p = 0.007). Sex, age, and the degree of valvar obstruction or regurgitation did not significantly influence the rate of progression of dilation. Conclusion: There is a significantly higher rate of dilation of the aortic root in adults with a bicuspid aortic valve when compared to the normal population. Periodic evaluation of the ascending aorta is essential in these patients, even after replacement of the aortic valve. Other imaging modalities should be considered if the region beyond the sinutubular junction is not well visualized by transthoracic echocardiography.

Aims: We investigated the incidence and outcome of cardiac malformations in 53 patients with Williams syndrome. Methods and results:The mean age, and period of follow-up, were 3.6 and 5.3 years, with standard deviations of 4.0 and 5.6 years, respectively. Of the patients, 45 (85%) had cardiovascular anomalies, often combined. Males presented earlier than females, at the ages of 2.1 years, with standard deviation of 2.8 years, as opposed to 4.5 years, with standard deviation of 4.2 years (p < 0.01). Supravalvar aortic stenosis occurred in 32 patients (71%), pulmonary arterial stenosis in 17 (38%), and mitral valvar prolapse in 12 (27%), 9 of these having regurgitant valves. Pulmonary valvar stenosis, ventricular septal defect, coarctation of the aorta, persistent patency of the arterial duct, hypertrophic cardiomyopathy, and subaortic stenosis all occurred less frequently. In 21 patients (47%), 24 surgical or catheter interventions had been made, most often for repair of supravalvar aortic stenosis, undertaken on 16 occasions with just one recurrence, and in 4 along with surgery to the mitral valve. Other lesions requiring intervention were pulmonary valvar stenosis, pulmonary arterial stenosis, coarctation of the aorta, and subaortic stenosis. We lost 3 patients (7%), with severe supravalvar aortic stenosis and moderate or severe mitral regurgitation, 2 early and one late after surgery. Conclusion: The most frequent cardiovascular anomalies in Williams syndrome were supravalvar aortic stenosis, pulmonary arterial stenosis, and mitral valvar prolapse, which occurred more frequently in our patients than previously observed. Patients with left ventricular pressure and volume overload were at greater risk.

A common brachiocephalic trunk is an anatomic variant in which both common carotid arteries and the right subclavian artery arise from the aortic arch via a single trunk. The impact of this condition on associated congenital cardiac malformations is presently unknown. Out of a total of 1480 cardiac catheterizations performed in children over a period of 10 years, we discovered 48 patients (3.2%) to have a common brachiocephalic trunk, of whom 98% had associated congenital cardiac malformations. A spectrum of associated lesions was identified, including left-to-right shunts in 19 patients, right-sided anomalies in 18 patients, left-sided obstructive lesions in 12 patients, and coronary arterial abnormalities in 10 patients, eight of whom had other cardiac defects. Genetic syndromes were present in one-fifth of the cases. When found with left-sided malformations, the common trunk was associated with persistent hypoplasia of the aortic arch, likely related to diminished flow through the arch during development. In each of four patients in whom the brachiocephalic trunk had been used during construction of a palliative shunt, we observed inadequate growth and deformation of the pulmonary arteries. Thus, angiographic identification of a common brachiocephalic trunk may be a marker for the presence of accompanying congenital cardiac defects and coronary arterial abnormalities. Understanding the pathophysiologic effects of the common trunk is important when planning the palliative or corrective procedures, and when assessing the potential benefit of the surgical repair over the long term.

In the Eisenmenger syndrome, indirect estimation of blood volumes may provide quite inaccurate information when seeking to define therapeutic strategies. With this in mind, we analyzed directly the red cell mass, plasma volume, and total blood volume in patients with pulmonary hypertension associated with congenital cardiac defects and erythrocytosis, comparing the results with the respective estimated volumes, and examining the changes induced by therapeutic hemodilution.

Thus, we studied 17 patients with the Eisenmenger syndrome, aged from 15 to 53 years, in the basal condition, studying 12 of them both before and after hemodilution. We also investigated five individuals with minimal cardiac lesions, aged from 14 to 42 years, as controls. Red cell mass and plasma volumes were measured using [51 chromium]-sodium chromate and [131iodine]-albumin respectively. Hemodilution was planned so as to exchange 10% of the total blood volume, using 40,000 molecular weight dextran simultaneously to replace the removed volume. The mean values of the red cell mass, plasma volume and total blood volume as assessed by radionuclide techniques were 32%, 31% and 32% higher than the respective volumes as estimated using empirical mathematical formulas (p < 0.002). The measured total blood volume was also 19% higher in the patients compared with controls. Following a period of 5 days after hemodilution, we noted a 13% reduction in red cell mass (p = 0.046), and 10% reduction in total blood volume (p = 0.02), albeit with no changes in the plasma volume.

We conclude that direct measurement of blood volumes is useful for proper management of these patients, and provides results that are considerably different from those obtained by empirical estimations.

Atherosclerotic disease of the heart likely has its origins in childhood. The promotion of cardiovascular health in children, however, has been studied only for those practicing in general paediatrics. We hypothesised that paediatric cardiologists do not consistently discuss cardiovascular risk factors with patients and their families. We therefore, carried out a nationwide survey of paediatric cardiologists to determine how often they discussed atherosclerosis and 4 modifiable risk factors, specifically weight, smoking, diet and nutrition, and physical activity. Only two-fifths reported that they discussed atherosclerotic disease frequently to always. For patients with cardiovascular disease, weight was discussed frequently to always by 59%, smoking by 61%, diet and nutrition by 63%, and physical activity by 92%. In contrast, for patients without cardiovascular disease, weight was discussed frequently to always by 35%, smoking by 46%, diet and nutrition by 39%, and physical activity by 62%. These differences are statistically significant (p < 0.003 by χ2 analysis). Cardiovascular risk factors were discussed more consistently as children grew older (p < 0.0001). Respondents stated that, in their opinion, the promotion of cardiovascular health was a role more appropriate for providers of primary care than for paediatric cardiologists (p < 0.0001). Constraints of time, and the perceived role of the cardiologist, were the most common barriers to anticipatory guidance. We suggest that these findings indicate that paediatric cardiologists can assume a more prominent role in preventive cardiology and education, although their precise role or roles, and the optimal methods of anticipatory guidance, remain controversial.

Our long-term follow-up has revealed that symmetrical development of the breasts is significantly impaired in pre-pubescent female patients subsequent to an anterolateral thoracotomy. Although standard posterolateral and anterolateral right-sided thoracotomies are used for correction of “simple” cardiac lesions such as patency of the arterial duct, coarctation of the aorta, or atrial septal defect, the required partial transection of large muscle groups, and injury to the developing tissues of the breast, may contribute to an unfavourable cosmetic outcome. Over the years, many surgeons, mostly specialising in thoracic procedures, have advocated mini- or axillary thoracotomies. In an attempt to improve surgical and cosmetic outcome, we have now adopted such a muscle-sparing approach, using a small horizontal midaxillary incision. We have now successfully employed the technique to close atrial septal defects in the oval fossa in 17 pre-pubescent females.

A man, aged 32, who had undergone surgical correction of complex congenital cardiac disease, had chronic severe right heart failure. Increasing reliance on diuretics led to deteriorating renal function, and he was rejected as a candidate for cardiac transplantation. By inserting a Tenckhoff catheter, and training him to drain his ascites at home, we achieved a dramatic improvement in both his cardiac and renal function. As far as we can ascertain, this is the first time this technique has been used for such a patient. We discuss the mechanisms of improvement, and suggest that other such patients with congenital cardiac disease might benefit from similar management.

Haemoptysis may occur in patients with tetralogy of Fallot and major aorto-pulmonary collateral arteries. We describe such a patient in whom bleeding from a major aorto-pulmonary collateral artery produced severe pulmonary haemorrhage. Interventional closure of the artery could not be performed because it perfused the native pulmonary arteries. Instead, we inserted a conduit between the right ventricle and the native pulmonary arteries, followed by percutaneous closure of the collateral artery. Our patient demonstrates the increasing necessity for combined surgical and interventional procedures.

Transcatheter techniques for occlusion of the persistently patent arterial duct using coils have become standard therapy at many centers for pediatric cardiology, and in selected patients have demonstrated comparable efficacy to surgical ligation. Surgical ligation may still be required in many cases, including premature infants or those born with low weight, those with ducts of large diameter, those with associated structural heart disease, and in circumstances of unsuccessful occlusion subsequent to attempted closure using coils. We report on the successful surgical ligation of an arterial duct of moderate size that exhibited residual patency despite two separate attempts at occlusion using coils.

Pulmonary oedema complicating balloon dilation of the pulmonary valve is extremely rare. We describe two children who developed acute pulmonary oedema soon after successful dilation of the pulmonary valve. They were treated with diuretics, ionotropes, and ventilatory support. Despite the supportive therapy, the complication proved fatal in one.

We describe a neonate presenting with a cholestatic jaundice due to Alagille syndrome. On echocardiography as part of the diagnostic work-up, we noted a slight dilation of the normally functioning left ventricle at the initial examination in the third week of life. Over the next 2 weeks, serial echocardiograms showed slowly progressive dilation and dysfunction of the left ventricle, together with a persistent mild elevation of levels of cardiac Troponin-I in the serum. These findings, unrelated to the underlying Alagille syndrome, prompted cardiac catheterisation, which confirmed that the main stem of the left coronary artery was originating from the pulmonary trunk. Surgery was successfully performed still in the presymptomatic period. The association of Alagille syndrome with anomalous left coronary artery arising from the pulmonary trunk is most unusual. We emphasise the cardiac findings prior to detection of the anomalous origin of the coronary artery.