In recent years, rheumatic fever has been declining in the industrialized countries, and has became a rare disease. In developing areas, nonetheless, the sequels of its cardiac involvement have important implications from the stance of public health, and this disease is still the main cause of heart disease in children and young adults. From a historical perspective, the long-term prevention and the control of socioeconomic problems have been proven to be effective, and it is these measures which have contributed to the decline of the disease in developed countries. But, due to its present high prevalence around the world, and the potential for his resurgence, this disease remains an unsolved problem. Furthermore, the complete understanding of its pathophysiology remains a challenge. In this presentation, we will discuss our experience with epidemiological, clinical and prophylatic aspects of this enigmatic disease, and the strategies we have developed to study and control heumatic fever in Minas Gerais, Brazil.

A patient with unrepaired complex pulmonary atresia had a normal life, achieving two successful pregnancies, until the age of 44 years. Confluent central pulmonary arteries were supplied by a fistu-lous communication from the left coronary artery, and from other collateral arteries arising from the underside of the aortic arch. Unusual aneurysms were present. Death at the age of 46 resulted from dissection and rupture of an aneurysmal dilation of the pulmonary trunk.

A clinical study on the outcomes of Glenn anastomoses performed since 1987 in eight consecutive patients aged ≥16 years, and in two performed earlier, showed poor results. One badly selected patient died early as a consequence of high venous pressure, while a further seven had early complications. Seven of eight hospital survivors were followed for 1–10 (median 4.2) years with two deaths (1 and 4 years later). Of the remaining five patients, two improved temporarily, but increased arterial oxygen saturation was not maintained after 6 months. The two patients who had undergone a Glenn anastomosis 10 and 34 years earlier were shown to have pulmonary arteriovenous fistulas. The Glenn anastomosis in these older patients is associated with high rates of complication and appears not to give adequate palliation, particularly when it is the only source of pulmonary blood supply. In the adult, the Glenn anastomosis can be used as a staging procedure for Fontan-type surgery, but must be combined with another source of pulmonary arterial supply. Any adult having a Glenn anastomosis, particularly without another source of pulmonary arterial supply, should be warned of the possibility of worsening of cyanosis and symptoms. The second stage of the procedure may need to be performed soon after the first should the hypoxia prove intolerable.

The acute complications of therapeutic cardiac catheterization for congenital heart disease as performed currently in a small unit were reviewed. In recent years, there has been a significant increase in the number of lesions thought amenable to catheter therapy. Only a few reports, however, have addressed the overall incidence of acute complications of therapeutic cardiac catheterization, all representing the experience of centres performing moderate-to-large numbers of procedures. A retrospective review was performed of 425 therapeutic catheter procedures performed at our institution between May 1993 and November 1997. Acute complications were retrieved from the database. This included all adverse events that were clinically recognized at the time of or within 2 weeks after the procedure and which, to the best of the authors' clinical judgement, were related to the catheterization and not part of the natural history of the child's illness. All patients were observed overnight following the procedure, and stayed in hospital if a complication developed. There were 49 acute complications (11.5%), of which 43 (10.1%) were deemed minor and 6 (1.4%) were considered major. The rate was low in patients with valvar pulmonary stenosis, including three neonates (3/45, 6.7%), for those undergoing angioplasty of native co-arctation (1/15, 6.7%) and pulmonary arteries (2/21, 7.4%); and for coil embolization of systemic to pulmonary collateral arteries (1/16, 6.3%). The rate was high in patients with valvar aortic stenosis, including 12 neonates (9/37, 24.3%), and for angioplasty of re-coarctation (4/23, 21.7%). There were more overall complications in neonates (25.6%) than in older patients (10.1%) (p < 0.01). Two patients died (0.5%), but no patient required emergency surgical intervention. In spite of the introduction of many new therapeutic modalities with greater intrinsic risk, and the fact that patients with more complex lesions and who are more acutely ill are being treated, the overall rate of complications remains relatively low. This probably reflects improvements in pericatheterization medical management, in selection of patients, in procedural techniques, and in the experience of operators.

The aim was to determine whether the clinical features of tachycardias originating from the right ventricular outflow tract in children with an apparently normal heart could predict the presence and the severity of the histopathological substrate. Thirteen children (median age 6 years; range 6 months–12 years) with tachycardia originating from the right ventricular outflow tract of apparently normal hearts, were assessed by echocardiography, heart catheterization with angiography, endomyocardial biopsy (13 patients) and magnetic resonance imaging (MRJ) (nine patients). Tachycardia was symptomatic in six and sustained in nine. Endomyocardial biopsy and MRI revealed acute myocarditis in five patients (38%), fatty infiltration of the right ventricle in two (15%), and minor histologic abnormalities in three (23%). Myocarditis was diagnosed in three of nine patients with sustained ventricular tachycardia, as opposed to two of four with non-sustained tachycardia (p = NS); in three of six symptomatic versus two of seven asymptomatic patients (p = NS); and in two of eight patients in whom ventricular tachycardia was induced during exercise testing as opposed to one of three in which it was not inducible (p = NS). A histopathological substrate was found in six of nine patients with sustained ventricular tachycardia, and in all four with non-sustained tachycardia (p = NS); in five of six patients with symptoms versus five of seven asymptomatic patients (p = NS); and in five of eight with inducible ventricular tachycardia during exercise testing versus all three in whom it was not inducible (p = NS). The mean rate of tachycardia was 184 ± 39 beats min−1 in patients with myocarditis, as opposed to 171 ± 48 in patients without myocarditis (p = NS); and 163 ± 33 in patients with a histopathological substrate compared with 210 ± 65 in patients without a histopathological substrate (p = NS). It is concluded that a histopathological substrate is present in the greater majority of children affected by the so-called right ventricular outflow tract tachycardia, but that the clinical features of the tachycardia do not predict the presence and the severity of this histopathological substrate.

The objective was to describe the epidemiologic, clinical, bacteriologic and therapeutic features of seven infants who developed sternal wound and mediastinal infections following palliation and/or repair procedures for congenital heart disease. A retrospective chart review was used. All infants with sternal wound and mediastinal infections were < 30 days of age at the initial operative procedure. Six of the infants had hypoplastic left heart syndrome, and one had complete transposition. Two infants required delayed closure of their chest wound. Three infants had superficial sternal infections and presented at a mean of 12 days postoperatively. Four infants had infection of the deep mediastinal structures: they were all asymptomatic and had purulent collections in their mediastinum at their second palliative operation, which was performed at a mean of 120 days after the initial surgery. Staphylococcus aureus, or coagulase-negative Staphylococcus, was isolated from the wound and/or blood of six infants. All infants with mediastinal infections were managed with operative debridement. Infants with superficial infections underwent local debridement. All infants received long-term intravenous antibiotics. Mediastinal infections in infants undergoing palliative staged procedures for congenital heart lesions may be chronic and indolent, resulting in delayed repair of congenital heart lesions.

Recent evidence has suggested that persistence of the left superior caval vein is associated with a high incidence of obstructive lesions of the left heart. To shed more light on this issue 1085 patients with congenital heart disease were studied retrospectively, with the aim of estimating the prevalence of a persistent left superior caval vein and its associated anomalies, focusing attention on obstructive lesions in the left and right ventricles. Patients with isomerism of the atrial appendages, or hypoplastic left heart syndrome, were excluded. A persisting left superior caval vein was present in 57 patients (5.2%). The overall incidence of obstructive lesions of the left heart was higher in patients with than in those without a persistent left superior caval vein (31.6 versus 7.8%, p < 0.001). Relative hypoplasia of the left ventricle was also higher in patients with persistent left superior caval vein (14 versus 0.8%, p < 0.001). The obstructive lesions found in the left heart, compared with the number in those without a left caval vein, were: mitral stenosis, 5.2 versus 0.7%; subaortic stenosis, 5.3 versus 0.9%; aortic coarctation, 17.5 versus 5.8% (p < 0.01); all of these in association, 3.5 versus 0.4%. In contrast, the incidence of obstructive lesions of the right heart was similar in the two groups of patients. It is concluded that persistence of the left superior caval vein can perturb the normal development of the left ventricle, being strongly associated with obstructions to left ventricular inflow and outflow.

We present the aims, methodology and initial results from the Italian Multicentric Study for the registration and follow-up of congenital heart disease. The general aims are to measure the prevalence of congenital heart disease in different geographic areas of Italy, and to assess the survival and outcome of affected babies. During the years 1992 and 1993, eighteen centers for Pediatric Cardiology spread all over the Country enrolled 1445 new babies with congenital cardiac malformations from a population of 341, 647 surveyed livebirths. The new cases were registered using the same methodologic criterions of the EUROCAT study in order to evaluate differences and/or similarities between the studies. The prevalence varied between 1.8‰ and 8.1‰; the average being 4.6%. The large range in prevalence is presumed to be related to different customs and hierarchies in flow and referral of patients. We provide total prevalence of individual lesions, and distribution of sentinel cardiac anomalies, in the Italian study and compare them with EUROCAT. Isolated ventricular septal defect is the most common lesion (39%); followed by atrial septal defect (7.5%); pulmonary valvar stenosis (7.3%); atrioventricular septal defects (5.4%); patency of the arterial duct (3.8%); complete transposition (3.7%); tetralogy of Fallot (3.3%); aortic coarctation (2.4%); aortic valvar stenosis (2.2%); and left heart hypoplasia (1.8%). The echographic stratification of ventricular and atrial septal defects, by location and size, was in keeping with the findings of the EUROCAT study. Because of the recent widespread availability of color-Doppler tecniques, the stratification of aortic and pulmonary valvar stenosis was an innovative approach in our study. Among the complex cardiovascular anomalies, double inlet ventricle and pulmonary atresia had a proportion of about 2% each; with double outlet right ventricle, common arterial trunk, Ebstein's malformation, tricuspid atresia, interrupted aortic arch and totally anomalous pulmonary venous connection having a proportion ranging from 0.5 to 0.8%. We discuss clinical features, such as frequency of extracardiac anomalies and familial aggregation of congenital heart disease, in comparison with the EUROCAT data.

Totally anomalous pulmonary venous connection to the azygos vein is a rare congenital heart malformation in which all the pulmonary venous blood returns anomalously to the azygos vein. Among 111 consecutive patients with totally anomalous pulmonary venous connection undergoing surgical correction at our institution between June 1982 and September 1997, this malformation was present in seven cases. By echocardiography, using a subxyphoid short-axis view at the atrial level and a modified suprasternal sagittal view, the malformation was diagnosed when the pulmonary venous confluence was traced posteriorly and superiorly relative to the right pulmonary artery and right bronchus, finally reaching reach the superior caval vein. Totally anomalous pulmonary venous connection to the azygos vein was misdiagnosed in the first two patients, both by echocardiography and angiocardiography. In the subsequent five patients, a precise diagnosis was obtained by echocardiography. Echocardiography, therefore, can be considered an accurate diagnostic tool permitting recognition of totally anomalous pulmonary venous connection to the azygos vein, and permitting corrective surgery without recourse to catheterization and angiography.

Isolated anomalous drainage of the right superior caval vein to the left atrium is a very rare cause of cyanosis in the newborn. Herein, the cross-sectional echocardiography and colour Doppler findings of this malformation are described.

A 28-year-old female patient with complete transposition, ventricular septal defect and persistence of the arterial duct underwent a palliative arterial switch procedure in 1976 at 7 years of age. Therefore, she has survived for 22 years and lives a near normal life. She is married, has been counselled against pregnancy and has increasing cyanosis with the typical features of the Eisenmenger syndrome.

An infant is described with congenitally corrected transposition and Ebstein's malformation. Banding of the pulmonary trunk had been previously performed because of a muscular ventricular septal defect. The patient underwent the double-switch procedure with the intention of unloading the morphologically right ventricle and the malformed tricuspid valve. This resulted in prompt postoperative functional and haemodynamic improvement.

A 3-year-old patient is described with an unusual form of co-arctation due to hypoplasia of the transverse arch and fibromuscular dysplasia involving a long segment of the thoracic aorta. Surgical repair required resection of the aorta from the distal transverse arch to the mid-descending thoracic aorta, and replacement with a 16-mm Dacron interposition graft. This case demonstrates the importance of preoperative evaluation of the entire aorta in the presence of co-arctation due to fibromuscular dysplasia.

The unusual case of an infant with aortic origin of the left pulmonary artery is presented. The patient developed a rare complication of lobar emphysema due to bronchial compression from the enlarged right pulmonary artery. Operative anastomosis of the left pulmonary artery to the pulmonary trunk was successful, with subsequent resolution of the lobar emphysema.

The rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. Cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation.

Congenital division of an atrial chamber is a very rare congenital malformation that more commonly affects the left atrium but which may, in rare circumstances, involve the right atrium. Such a divided right atrium may present with symptoms consistent with increased portal venous pressure. Reported is a case with unusual clinical presentation. The patient underwent resection of the dividing shelf with good postoperative results.