We have conducted, in two stages, a descriptive and comparative analysis of the diagnostic and evolutionary clinical aspects of rheumatic fever. The descriptive analysis was based on a sample of 226 patients seen between 1976–1986. The forms and frequency of the manifestations of rheumatic fever were studied, as well as the difficulties encountered in diagnosing mild cases and the risk of making a hasty diagnosis due to the high prevalence of the disease. Subsequent to the establishment of an outpatient clinic for referral of patients with rheumatic fever, as part of a study and control program for the disease, 98 patients were seen in the period 1988–1991. In order to compare the profile of the manifestations in the acute phase with evolutionary aspects over the course of the disease, a subgroup of 61 children, treated in the period 1983–986 was selected to obtain equivalence in size of sample and time of follow-up (p = 0.08). The comparative analysis revealed that no modifications have occurred over the period of study in the profile of the manifestations during the acute phase, and no statistically significant differences have occurred in age at first attack, severity ofcarditis, or in the type ofvalvar and articular involvement. Evolutionary data after rigid control of primary and secondary prevention, however, showed a marked reduction in the incidence of recurrence (p = 0.036), the frequency of hospital admission (p = 0.001) and deaths (p = 0.024). The main factors modifying the evolution of the disease were analyzed. The action of the health team had a fundamental importance in the control of recurrence. This fact is especially important for developing countries, where a reduction of the prevalence of the disease can only be achieved through major structural socioeconomic changes in the community.

M-mode, cross-sectional and color flow Doppler echocardiography were performed in 28 patients with acute rheumatic fever. The patients were grouped according to the presence or absence of carditis and congestive heart failure. Abnormal echocardiographic findings were found in 25 patients (89%), including five with no carditis. M mode echocardiography showed significant cardiac enlargement in all patients with carditis, and in two patients with no carditis. The myocardial contractility, as based on ejection fraction and fractional shortening, was normal in all patients except one. Prolapse of the leaflets of the mitral valve (57%) and increased echogenicity of the leaflets of the mitral valve (36%) were the most common findings seen on cross-sectional echocardiography. Pericardial effusion was seen in six patients (21%), all with heart failure. Color flow and Doppler echocardiography showed that mitral regurgitation, which was seen in 24 patients (86%), was the most common finding. Aortic regurgitation was found in 17 patients (60%). The presence of congestive heart failure in patients with carditis was related to the severity of the valvar regurgitation and the number of valves involved. Echocardiographic evidence of rheumatic involvement of the heart may be observed even in the absence of clinical signs of carditis.

We studied 56 children echocardiographically, ages four to 15 years (mean nine years) with acute rheumatic fever to determine the extent of cardiac involvement. All had a clinical diagnosis of carditis. Mitral regurgitation was always present and was associated with aortic regurgitation in 53.6% and tricuspid regurgitation in 32.1%. Mitral valvar prolapse was observed in 18 patients 5(32.1%) and could not be differentiated from myxomatous prolapse in 10 (17.8%). Cordal rupture was detected in seven patients (12.5%), three of whom required surgical treatment. Other findings concerning the mitral valve were vegetations in two patients (3.6%), commissural fusion without stenosis in 36(64.3%), and mitral stenosis in four (7.1%). Heart failure was usually secondary to valvitis rather than myocarditis, and led to surgical treatment in seven patients. The myocardial function was depressed in only two patients. In both, the ejection function returned to normal after medical treatment. Echocardiography provides important information on the involvement of the heart in acute rheumatic fever, helping to determine prognosis and the results of treatment.

Eight patients, four males and four females, age five to 13 years old (average: 11 years) have undergone surgery in the acute phase of rheumaticfever. The patients presented a history of rheumatic activity characterized by the presence of migratory arthritis and carditis. All patients had severe acute mitral insufficiency, while one of them had associated aortic insufficiency. Laboratory examinations revealed the presence of an acute inflammatory condition. All patients had acute heart failure and were treated initially with high doses of diuretics, peripheral vasopressor and vasodilator amines, together with cardiotonic drugs, without improvement. Surgical treatment was indicated after a period of observation between 24 hours and five days. In five patients, the Doppler echocardiogram revealed rupture and elongation of tendinous cords. Two of them had acute dilatation of the mitral ring, and one had isolated acute dilatation of the mitral ring. Five patients underwent valvar replacement and, in three, valvar repair was carried out. Two patients, who were in cardiogenic shock at the time of their referral, died in the operating room following replacement of the mitral valve. All patients who underwent repair of the mitral valve were in good condition at the last follow-up, six to 27 months after surgery.

Although declining in the so-called “first world,” surgery for rheumatic valvar lesions in patients less than 16 years of age is still a challenging problem in lesser developed countries. From 1970 to 1990, we operated upon and followed-up 136 children (less than 16 years) with different valvar lesions, performing various surgical procedures. The mitral valve was most frequently affected (52.9%) followed by multiple valvar disease (39.7%) and aortic (7.3%) involvement. The operations performed were valvoplasties (67), valvar replacement (49), and combined procedures (20). Hospital mortality for the overall group was 10.2%. There were 71 reinterventions in the follow-up of 20 years. Actuarial survival was 60.3% including hospital mortality. The event-free actuarial survival showed that 91% had at least one major event which required reoperation or hospital treatment, or else invasive cardiac procedures. We conclude that rheumatic valvar disease is still a challenging problem in our country, and that the severity of the illness is directly related to the younger age patients. In spite of reasonable long-term survival, very few patients had an uneventful follow-up after surgery.

A total of 246 consecutive patients were seen with the diagnosis of acute rheumatic fever (and/or rheumatic heart disease) and were followed for 587.7 patient years. The episode of acute rheumatic fever was the first in 64 of these patients, whereas recurrent acute rheumatic fever was seen in 26 and the other 156 patients had chronic rheumatic carditis. At presentation, those suffering an initial attack had less frequent and less severe carditis when compared to those suffering recurrent infection (p<0.05). Improvement in carditis during follow-up was noted in those having an initial attack (p<0.1), while deterioration occurred following recurrent infection (p<0.01), and no change was noted for those with chronic infection. Recurrences of acute rheumatic fever were most frequent in those presenting with their initial infection (21%) or reinfection (35%), and dropout from follow-up was highest in the group with first infection (38%) compared to those with recurrent infection (15%) and chronic carditis (25%). Non-recognition of the first episode of acute rheumatic fever and failure of secondary prophylaxis were found to be the major contributors to the observed increased pool of recurrent and chronic rheumatic heart disease. We conclude that, in the absence of programmed primary prophylaxis of acute rheumatic fever, the best chance of controlling the progression of carditis or affecting cure is to recognize the first episode ofacute rheumatic fever and then ensure strict adherence to secondary prophylaxis. Since the prognosis of recurrent carditis is poor, the best management of moderate to severe recurrent carditis is early reparative valvar surgery wherever possible.

We reviewed 2,756 necropsy studies of patients who died because of rheumatic heart disease. The anatomic material was divided into two groups. The first group was made up of 404 cases (14.6%) having the association of rheumatic and congenital heartdisease. The second group comprised 2,352 cases with isolated rheumatic heart disease. The analysis of the site and types of congenital malformations in the first group showed 160 cases (39.6%) with aortic, 112 (27.7%) with pulmonary, 15(3.7%) with tricuspid and six (1.5%) with mitral lesions. Twenty patients (4.9%) had an atrial septal defect whilenine (2.2%) had patency of the arterial duct and four (1 %) a ventricular septal defect. The incidence of rheumatic aortic valvar disease in cases also having congenital lesions of the aortic valve (83.7%) was higher than in patients with isolated rheumatic involvement of the aortic valve (67.7%) (p<0.001). The difference in the incidence of rheumatic lesions found in patients with congenital pulmonary lesions (9.8%) and in the patients with isolated rheumatic disease (3.3%) was also significant (p<0.001). No differences were observed in rheumatic lesions to the mitral and tricuspid valves in those with or without associated congenital lesions. In patientswith an atrial septal defect, there was a higher incidence of combined mitral and tricuspid lesions (35% vs. 18%, p<0.02). Patients with patency of the arterial duct andco-existing rheumatic heart disease had a greater incidence of isolated rheumatic aortic disease (11% vs. 0.7%), and also of combined aortic and mitral lesions (33% vs. 17%), but the differences were not significant. The incidence of involvement of all four valves was 5.4% in those with associated congenital lesions and 3% in those in which rheumatic involvement was isolated (p<0.O2). We conclude that rheumatic heart disease has a tendency to involve preferentially cardiac valves that are the sites of congenital malformations.

We have investigated the hearts from 153 infants found dead in their cots at ages ranging from one month to one year. The deaths occurred over a period of five years (January 1986 to December 1990) and were studied in a center for the study of the sudden infant death syndrome located in Paris. The epidemiological characteristics of this group are:male predominance (sex ratio 1.43), age less than four months (82%), and a predominance of winter deaths. These are the characteristic features ofthe sudden infant death syndrome. Ofthe 143 children studied with the permission of their parents, 24 (16.8%–12 girls and 12 boys) had histological lesions consistentwith myocarditis according to the Dallas criteria. The histological diagnosis of myocarditis is based on the association of cellular necrosis with a mononuclear or mixed inflammatory infiltrate. Cytoplasmic vacuolization, the presence of inflammatory cells in myocytes, and rupture of the cell walls of myocardial fibres are the equivalent histological signs of cellular necrosis. Myocarditis was diversely associated with respiratory, hepatic, muscular, gastrointestinal and/or neurological involvement. Twelve infants had previously been ill. Two died during the course of a serious illness. In only four cases was a viral association demonstrated. This incidence of myocardial involvement, similar to thatdescribed elsewhere in the literature, suggests that myocarditis could be a pathogenic explanation of some deaths thought on general autopsy investigation to be sudden and unexplained.

There is a consensus that sudden infant death syndrome, the leading cause of mortality in the first year of life, is multifactorial. Most of the cases are probably due to respiratory or cardiac disorders. It has been proposed that some cases of sudden infant death might result from ventricular fibrillation triggered by a sudden increase in sympathetic activity affecting the heart with reduced electrical stability. This impairment can be due to an insufficient or delayed development of cardiac vagal innervation, with the resultant lack of its protective effect that has been demonstrated to be present since the third week of life. Clinical studies suggest that some of the sudden infant death victims may have a reduced heart rate variability that could be interpreted as a decreased parasympathetic activity to the heart. On the other hand, a reduced cardiac electrical stability may be provoked by a developmental imbalance in sympathetic innervation such to create a dominance of left-sided nerves. We have demonstrated that an imbalance of this type experimentally induced in puppies increases the susceptibility to ventricular fibrillation and prolongs QT interval. In order to demonstrate a significant relationship between prolonged QT interval and sudden infant death, we performed a large prospective study on 18,832 neonates. QT interval corrected for heart rate (QTc) measured from an electrocardiogram on the fourth day of life, was within the normal limits in nine victims from known causes, while it was exceeding the mean by two standard deviations (>433 msec) in six of 12 sudden infant death victims. If these data are confirmed by a larger multicenter study started in Italy, it will be possible to identify some of the sudden infant death victims by the observation of a prolonged QT interval on the electrocardiogram. In this case a preventive strategy based on a time-limited administration of beta adrenergic blocking agents to the infants at risk, might reduce the incidence of sudden infant death.

In this prospective study, pulmonary blood flow was measured using transesophageal Doppler echocardiography to assess whether ventilation by means of external high frequency oscillation around a negative pressure baseline can increase pulmonary blood flow, compared to intermittent positive pressure ventilation, in five patients after the Fontan operation. Pulmonary blood flow was measured when patients were ventilated by means of intermittent positive pressure ventilation and again during equivalent negative pressure ventilation using the external oscillatory technique. When compared to that with intermittent positive pressure ventilation, ventilation using external high frequency oscillation increased pulmonary blood flow by 116 ±61.5% (p=0.013). These results show that ventilation using an external oscillatory device with a mean negative chamber pressure may provide hemodynamic advantages in patients requiring assisted ventilation after the Fontan operation.

Obstruction of the left ventricular outflow tract continues to represent a challenging surgical problem, particularly in children. Failure to relieve the obstruction by either valvotomy or myectomy requires enlargement of the ventriculoaortic junction and outflow tract. Alternatively, the obstruction can be by passed using an apical valved veritriculo aortic conduit, althoughthis is associatedwith a high rate oflong-term complications. The use of a small xenograft in children is less than ideal because of early calcification, and the insertion of a mechanical valve may be feasible only after considerable enlargement of the ventriculoaortic junction. Aortoventriculoplasty is particularly suited for this purpose, and it is usually associated with good early results. Yet, long-term experience with this procedure, as far as we know, has not been reported previously. We describe our experience with the use of aortoventriculoplasty in four patients, three of whom have been followed-up for more than five years. Two patients have required reoperation for replacement of their prosthetic valves, one for bacterial endocarditis and the other for fracture of a continuous prolene suture which secured the prosthetic valve to the Dacron septal patch. All patients are currently in good health with continuing satisfactory relief of obstruction to their left ventricular outflow tract.

Non-surgical occlusion of the arterial duct (ductus arteriosus) has been shown to be effective. Its limitations are yet to be fully defined. We have attempted to elucidate this issue in the setting of our early experience with the procedure, utilizing the Rashkind double disc occluder. After clinical and echocardiographic evaluation, 56 consecutive patients were studied invasively. In two patients, the patent duct was considered too large for occlusion and in one patient it could not be crossed with the diagnostic catheter. In the remaining 53 patients (95%), an attempt to implant the double disc occluder was made. The mean age of all patients was 7.9 years (range 0.16 year to 29 years). There were 35 females (63%) and 21 males. The mean weight was 24.2 kg (range 6.7 kg–85 kg). In five patients (9.5%), the procedure failed. This was due to pull through of the 17 mm device in three patients. In another patient, because of a very small duct (post surgical ligation), it was not possible to deliver the distal legs of the 12mm device. In the last patient, the device embolized to the left pulmonary artery and was retrieved to the groin. Subsequent replacement was not attempted. In 48/53 patients (90.5%) the device was successfully implanted. Nine cases of the 48 in whom the device was implanted (18.8%) showed complete occlusion at 10 minutes and 22 more (45.8%) five to 24 hours after the procedure. A total of3 1/48 patients (64.5%) were discharged home the day following the procedure with no residual shunt, as judged by color flow Doppler studies. The mean duration of the procedure in all patients was 94 minutes (range 47 to 244). The fluoroscopy time ranged from 3 to 81 minutes with a mean of 17 minutes. Follow-up data (between five days and 18 months) are available in 20/48 patients. Three patients had a clinically detectable residual shunt, with an audible continuous murmur six months after the procedure. All three have undergone complete occlusion using a second device. One more patient showed spontaneous occlusion on follow-up. So far, a total of 35/48 patients (72.9%) have had complete occlusion of the patent duct. We conclude that not all patients with an arterial duct are amenable to occlusion using the Rashkind device. The procedure appears to be safe when performed by a suitably trained novice, nonetheless, and successful implantation can be achieved in over 90% of patients.

This study aimed to assess the prevalence of “silent” valvar regurgitation in healthy Brazilian children; to determine whether variations in the gain settings of the machine interfered with its documentation; and to compare Doppler color flow mapping with the pulsed wave Doppler. Twenty-five healthy children were selected randomly. Echocardiographic equipment used was ALOKA 870. Fixed parameters were 3.5 MHz transducer, 400 Hz filter, reject of 7/14 and aperture of 30/80. Variable parameters were Doppler color flow mapping and pulsed wave Doppler gains. A protocol for evaluation of each cardiac valve was developed. Regurgitation was noted in 93% for the pulmonary valve, 74% for the tricuspid valve and 27% for the mitral valve. In the aortic valve, jets suggestive of mild valvar regurgitation were observed with the Doppler color flow mapping technique in two children but these findings were not corroborated by pulsed wave Doppler. Only in the aortic valve were findings from Doppler color flow mapping not in agreement with those from pulsed wave Doppler. Variations in the settings for gain did not interfere significantly in the documentation of regurgitation. Inter-observer variations were not significant (two independent operators). The clinical implications of these findings are yet to be established. Labeling these regurgitant jets as “normal” may be oversimplifying their meaning and perhaps overlooking the possibility of predisposition to endocarditis or progression of the valvar abnormality. Conversely, labeling them as “abnormal” can generate a more serious problem, which is that of iatrogenic heart disease. Prospective follow-up studies should be carried out in these subjects.

A complex malformation of the heart was diagnosed by fetal echocardiography at 27 weeks of gestation. The study of the relationship of the abdominal great vessels, showing the inferior caval vein anterior and to the left of the aorta, made possible the diagnosis of right isomerism. This inferential guide to the arrangement of the thoraco abdominal organs proves to be as valid in the fetus as it is in the neonate and child. The examination of the abdominal great vessels as part of the fetal scan is of great value, enabling us to counsel mothers appropriately in the light of the poor prognosis of neonates with isomerism of atrial appendages and complex intracardiac lesions.