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We surveyed the incidence of congenital heart disease in 49887 native live born children in the period between 1984 to 1994 in Qatar. Each child with clinically suspected congenital heart disease underwent echocardiographic examination. Magnetic resonance imaging, cardiac catheterization and surgical intervention were done at the discretion of the patient's pediatric cardiologist. Virtually no postmortem examinations were performed. Children with congenital heart disease were entered into a computerized database and were then followed for 1-11 years. Congenital heart disease was diagnosed in 610 of 49,887 children for an incidence of 12.23/1000 live births. The reasons for the high incidence were high proportion of small muscular ventricular septal defects discovered before the time of their spontaneous closure, referral to and follow up by a single group of pediatric cardiologists, location of the pediatric cardiology service in the same setting where nearly all of the deliveries took place, freely available health care service, and echocardiographic examination of every child with a clinical diagnosis of congenital heart disease.

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.

Objective: To determine the frequency of occurrence of mitral and aortic valvar regurgitation in rheumatic children in whom there was no evidence of carditis acutely or at an earlier attack., Design: Colour flow Doppler imaging was used in a non-randomised study of sequentially admitted children who met the criteria for acute rheumatic fever without clinically evident carditis and patients in whom the disease was quiescent after a previous attack of rheumatic fever. Two separate control groups were used for comparison of the echocardiographic findings, and a group of patients with confirmed rheumatic carditis was included for comparison of acute phase and antistreptococcal reactants., Setting: A general hospital with the only paediatric inpatient department in Qatar., Patients: From November 1988 to October 1990, 11 children were studied during the acute rheumatic period. In seven additional children the disease was quiescent when they were studied 18 to 36 months after a documented episode of acute rheumatic fever in which there was no evidence of carditis. The control patients were all studied during the same period., Main Outcome Measure: Detection of mitral and aortic regurgitation in patients without clinical evidence of rheumatic carditis in the acute or quiescent stages of the disease., Results: Mitral or mitral and aortic regurgitation was found in 10 of the 11 children studied in the acute rheumatic period. None had a murmur or other evidence of carditis. In all the cases studied the valvar insufficiency was mild. Four of the children studied late in the quiescent period had either aortic or mitral insufficiency by colour flow Doppler evaluation; two children who had previously had valvar insufficiency no longer showed this, and one child without positive findings in the acute phase remained without insufficiency. None of the non-rheumatic control subjects showed mitral or aortic regurgitation., Conclusions: Colour flow Doppler imaging is a useful method of identifying subclinical mitral and aortic valvar disease at all stages of rheumatic fever when carditis cannot be otherwise detected and is a valuable addition to current diagnostic criteria.

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.

Thirteen patients undergoing complete evaluation for double-chambered right ventricle were subjected to phonocardiographic evaluation. Each of these individuals revealed a holosystolic ejection-type murmur which exhibited a similarity of appearance unaltered by the degree of physiologic derangement or associated cardiac abnormalities. This finding is considered to be caused by the obstructive elements in double-chambered right ventricle and appears to be a characteristic finding for this condition.

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.

M-mode echocardiographic features are presented in four patients with membranous ventricular septal defect. The most consistent echo findings in all the patients were the presence of fuzzy, fluttering echoes of the tricuspid valve during systole. More careful echocardiographic evaluation of the tricuspid valve in ventricular septal defect is therefore recommended.

Thirteen children and young adults with coarctation of the aorta as their principal cardiovascular abnormality, 11 with bicuspid aortic valves, were evaluated by orifice-view aortography to evaluate their aortic valvular morphology. For comparison 30 individuals with aortic valvular deformities but without coarctation of the aorta were similarly studied. Two distinct forms of bicuspid valves could be identified characterized by either the appearance of gross inequality of size of the two valve leaflets or an appearance wherein each leaflet closely approximated the size of the other, thus equally bicuspid. Excepting two individuals with normal, tricuspid, aortic valves, all of the patients with coarctation of the aorta had equally bicuspid aortic valves which contrasted to the group without coarctation in which the unequally bicuspid type predominated. This difference in bicuspid aortic valve morphology associated with coarctation of the aorta suggests a different developmental process involving the aortic valve as opposed to the situation in individuals without coarctation.

The case of a newborn who had an umbilical catheter inserted in the intensive care unit is discussed. This catheter was meant to be inserted into the umbilical artery, but was instead inadvertently inserted into the umbilical vein. It crossed a patent foramen ovale into the left atrium. This fact was not known at the time of echocardiographic evaluation. At echocardiography, abnormal echoes within the left atrium were seen. Findings were typical for a catheter within the heart, and the umbilical catheter was subsequently withdrawn. There is a potential for mistaking the echocardiographic findings for a left atrial abnormality and echocardiographers should be alert to this possibility.

Three newborn infants were observed to have murmurs restricted to diastole. None was considered ill, and none has exhibited symptoms or findings of cardiopulmonary disease throughout follow-up. Localization of the diastolic timing and absence of a significant systolic component of the murmur were confirmed by phonocardiography. The transient nature of the finding was established by serial phonocardiography. Essentially normal cardiac anatomy was determined by routine chest radiograms, electrocardiograms, and echocardiography in both M and two-dimensional modes. Suggestive evidence for left-to-right shunting was present in each case. Invasive studies were not performed. An anatomic cause for the diastolic murmur was not discovered, suggesting it could be related to the ductus arteriosus or mild pulmonary insufficiency.

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.

This report describes a case in which a persistent third aortic arch connection partly palliated interruption of the aortic arch. The malformation occurred without other cardiovascular abnormality in a newborn infant in whom the principal diagnostic clinical finding was the absence of all peripheral pulses except for those of the right brachial and carotid arteries. Echocardiographic and angiocardiographic examination showed a continuation of the left internal carotid artery to the descending aorta by way of an arching communication.

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.

Differently prepared aortic root allografts were implanted in the right ventricular outflow tract of growing puppies to determine the site of origin and progress of degenerative changes in these conduits. The three preparations assessed were as follows: group A, fresh and sterile grafts; group B, antibiotic sterilized grafts in nutrient medium; and group C, beta-propiolactone sterilized grafts. Although calcification of the aortic wall occurred in all groups, the aortic leaflets were minimally affected. A correlation between viability and lack of calcification and between viability and long-term function is emphasized.

The identification of absence of continuity of the inferior vena cava (IVC) with the right atrium assumes major importance at the time of right heart catheterization. The appearance of the IVC in the lateral chest radiogram is diagnostically different from the normal when discontinuity is present and 2 radiographic patterns have been recognized: 1) total absence of the IVC shadow; 2) straightened and posteriorly directed IVC shadow contrasting sharply with the anteriorly directed normally continuous IVC. This latter sign is considered reproducibly accurate in the identification of the IVC which fails to have continuity with the right atrium.

Of 12 patients who underwent complete hemodynamic and cineangiocardiographic study for double-chambered right ventricle, seven were found to have a previously undescribed abnormality. Each of these patients exhibited an actively contractile, pouchlike structure closely associated with the right ventricular outflow tract, which occupied a right medial position in respect to the right ventricle. It varied considerably in size but not in location. This abnormality could be best defined when the distal, low-pressure, aspect of the doubled-chambered right ventricle was the injection site. It was best seen in those angiographic projections which allowed optimal viewing of the right medial aspect of the right ventricle. This structure is considered to develop postnatally, secondary to altered flow patterns produced by the underlying malformation, and it appears to constitute a unique finding with double-chambered right ventricle.

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.

Orifice-view aortography is a contrast cineaortographic technique that allows en face viewing of the aortic valve. In this projection the anatomic configuration of the aortic valve is identified, and it is possible to planimeterize the visualized aortic valve opening and accurately determine the orifice area. Fifteen individuals with congenitally deformed aortic valves with gradients ranging from trivial (< 10 mm Hg) to surgically significant (6E 60 mm Hg) were subjected to aortic orifice area measurement by orifice-view aortography, and the area was compared with the peak systolic gradient and valve area. The results yielded a cloe correlation with the aortic valve area index as measured hydraulically or, as in three instances, directly from the surgically excised specimen (r = 0.94). A satisfactory correlation was also shown with the valve area index and the peak aortic valve gradient (r = 0.85). These observations indicate that orifice-view aortography is an accurate method for the measurement of the aortic valve orifice and may be particularly useful when hydraulic measurements are unattainable or invalid.