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An eleven-year study of the incidence and consequences of acute rheumatic fever was carried out in a country in which a uniform climate together with national characteristics of insularity, wealth, and unrestricted access to free medical care contribute prominently to the epidemiologic milieu. Study subjects were 86 children, aged four to fourteen years, satisfying criteria for acute rheumatic fever. Study methods included clinical evaluation, standard laboratory studies, and echocardiography. A declining incidence of rheumatic episodes, ranging from 1.06 to 18.6/100,000 population (average 11.2/100,000), was identified. The course of the episode was generally mild. Arthritic findings predominated (92%), followed by carditis identified clinically in 43% and, with the addition of echocardiography, in 71%. Residual valvular regurgitation, as a longer term consequence, persisted in 46% of those with auscultatory confirmation of valvulitis. No recurrences were identified. Comparison with countries of similar socioeconomic status revealed relatively unimportant differences. Comparison with nearby disadvantaged countries identified striking contrast. It may be concluded that among the contributing factors, for the improvement in the incidence and sequelae of a rheumatic episode, are an advantaged socioeconomic environment and accessibility to unlimited medical care.

A four-and-a-half-month-old infant suffered sudden circulatory collapse early in the course of a brief febrile illness. The electrocardiogram initially appeared normal; an abrupt change indicative of extensive myocardial infarction occurred following stabilization. Coronary arterial anatomy and flow by echocardiography and angiography were normal except for the finding of single origin of the entire coronary system from the left sinus of Valsalva. Gallium 67 myocardial imaging was positive for an inflammatory process, and the diagnosis of myocarditis was supported. This is the first case, to the authors' knowledge, of myocarditis simulating myocardial ischemia in an infant of an age in which anomalous origin of the left coronary artery from the pulmonary artery is a recognized cause for myocardial infarction.

Twenty-two cases of double-chambered right ventricle studied in detail from two different centers comprise the study population. Of these, 5 have trisomy-21 Down's syndrome; these five cases, comprising nearly 25% of the entire study group, were essentially evenly divided between the two centers. This unexpectedly high percentage of Down's syndrome associated with such an unusual cardiovascular malformation, albeit among a small population, is likened to the currently recognized and similar association of Down's syndrome and atrioventricular cushion deformities.

A detailed clinical analysis of 16 patients with various forms of congenital cardiac malformations with the potential for large left-to-right shunts who developed extreme pulmonary hypertension in infancy and early childhood has been carried out. The study plan was to include only patients with sufficiently long follow up to allow analysis of their course in both the prepubescent and postpubescent periods. In early life these patients were all characterized by lack of evidence for significant development of the expected left-to-right shunt indicating inappropriate maturation of their pulmonary vascular circulation. Throughout all phases of their life, they were significantly polycythemic in comparison with a control group but this polycythemia, representative of peripheral systemic hypoxemia, increased markedly with the acquisition of puberty in all patients. These two findings indicate both an early abnormality, suggestively primary, of the pulmonary vasculature and a late progression of the disease associated with pubertal maturation.

The association of a bicuspid aortic valve with other congenital cardiac malformations, prominently coarctation of the aorta, is widely accepted with estimates of frequency reported to be 20% overall and ranging between 25-50% to as high as 85% when there is coarctation. The anatomic configuration of the valve has received little comment and, excepting those individuals requiring aortic valve surgery coincident with repair of other malformations, has been limited to postmortem examination. Recently, however, an angiographic technique for accurately evaluating the morphology of the aortic valve has been utilized allowing for the determination of aortic valvular appearance at routine evaluation by cardiac catheterization. We have currently examined 17 individuals with various forms of congenital cardiac malformations in whom an aortic valvular abnormality was suspected as a coexistent abnormality employing the technique of orifice-view aortography. The present report concentrates on the aortic valvular anatomy of these patients.

The child with significant valvular heart disease may present in any single instance both the most difficult of management decisions or a surprisingly simple one. Thus, if a direct surgical approach on the valve is feasible, the problems may very well be quite simple and straightforward; conversely, if valve replacement is indicated such may not be the case. We have recently reported our experience with one type of valvular heart condition, congenital aortic stenosis, in respect to determination of surgical approach based upon the ability to predict the orifice area and valvular anatomy. This report reviews our current experience with this technique. As well, the experience concerning the approach to each of the valvular conditions will also be reviewed with emphasis in respect to the left heart valves and particularly their long term postoperative management.

The purpose of this investigation is to demonstrate the potential diagnostic value of intracardiac sound recordings in patients with subaortic stenosis. Intracardiac pressure and sound were measured in 10 patients with various types of subaortic obstructions using a catheter-tip micromanometer. Seven patients had idiopathic hypertrophic subaortic stenosis (IHSS), 2 had a subvalvular membrane, and 1 had a subvalvular tunnel. Within the left ventricular cavity, at the site of maximal systolic left ventricular pressure, either there was no systolic murmur, or the murmur was of low intensity. However, within the outflow tract of the left ventricle, distal to the site of intraventricular obstruction, a prominent systolic murmur was detected in all patients. This murmur was of higher intensity than the one measured distal to the aortic valve. In one patient, in whom no subvalvular obstruction was present, but in whom entrapment of the tip of the catheter occurred, no murmur was detected in the left ventricle even though a subvalvular pressure gradient was observed. Therefore it appears that a systolic murmur recorded with maximal intensity in the outflow tract of the left ventricle may be of substantial help in distinguishing between an artifactual intraventricular pressure gradient, and one that results from intraventricular obstruction.

A child with a bronchogenic cyst is described who manifested clinical findings indicative of pulmonary arterial stenosis. Compression of the right pulmonary artery by the cyst with a significant pressure gradient between the proximal and distal segments of the right pulmonary artery was verified at surgery. The post-operative follow-up findings have confirmed relief of the stenosis with absence of the murmur originating from it.

One hundred twenty-four adult patients consecutively referred for suspected interatrial septal defect (IASD) were studied with contrast echocardiography. In 58 the study eliminated altogether the diagnosis of any significant cardiac defect. Fifteen patients had other forms of cardiac abnormalities with no shunting of blood, and 51 patients had echocontrast study positive for interatrial shunting; 48 of these were subsequently proven by cardiac catheterization to have IASD. Echocontrast provided a highly sensitive and specific means of both diagnosing IASD and eliminating this diagnosis.

Hypoplasia of the right ventricle occurring as an isolated abnormality is an uncommon cause of cyanotic congenital heart disease. Its presentation in the newborn may mimic the ductus dependent and less stable right ventricular hypoplasias such as tricuspid atresia and pulmonary atresia. However, the natural history of this abnormality contrasts with those ductus dependent lesions by the rather benign course it presents. A patient with only marked reduction in right ventricular size and commensurate reduction in size of the tricuspid and pulmonary valves is described. Although cyanotic this child has shown none of the critical problems frequently encountered with the more severe forms of the right ventricular hypoplasias.

The M-mode echocardiogram is considered a satisfactory diagnostic tool for the determination of the presence of a bicuspid aortic valve. M-mode echocardiographic failure to determine this condition is occasionally encountered and has been suggested to be due to the anatomic configuration wherein the two aortic valve leaflets are nearly equal in size. By employing a specific angiographic study (orifice-view aortography), it has been shown that accurate identification of aortic valve anatomy is possible. Ten patients having orifice-view aortographic findings of an aortic valve having two essentially equally sized leaflets have been studied both by this technique and M-mode echocardiography. Five of these had M-mode echographic evidence of a bicuspid aortic valve. However, 3 exhibited no criteria diagnostic of a bicuspid valve and 2 had only suggestive criteria. The results confirm that M-mode echocardiography alone is insufficient to determine reproducibly the presence of an aortic valvular malformation with or without stenosis when the anatomy is that of an equally bicuspid aortic valve.

Infants with the hypoplastic left heart syndrome present a reproducible appearance that terminates in irreversible shock during the neonatal period. There is disagreement over the exact cause of this appearance. This case involves a newborn infant with tricuspid atresia and transposition of the great arteries, and with the clinical appearance and course usually reserved for neonates with the hypoplastic left heart syndrome. Accurate diagnosis is crucial in all such infants to correctly distinguish those with a hopeless outcome from those amenable to palliative cardiovascular surgery. This case supports the contention that ductal closure alone is not the principal cause of death in infants with hypoplastic left heart syndrome.

The presence of a specific deformity of the right ventricle in association with the double-chambered right ventricle (DCRV) has been discovered in 8 of 14 individuals undergoing detailed study for DCRV. Investigational study employing multiple angiographic projections with selective angiography has allowed precise morphological identification of this deformity as a contractile pouch located arising from the outflow tract of the right ventricle. Comparison of the angiographic data with the electro-vectorcardiographic findings of DCRV suggests that these highly specific electrical features of DCRV correlate with the position and location of the contractile pouch.