Acute rheumatic fever in an Arabian Gulf country--effect of climate, advantageous socioeconomic conditions, and access to medical care.

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.

Double-chambered right ventricle and Down's syndrome: a proposed new association.

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.

Double outlet right ventricle associated with cor triatriatum sinistrum.

Echocardiographic examination of a newborn infant showed a double outlet right ventricle with subpulmonary ventricular septal defect. The left atrium was divided by a membrane-like structure into a proximal chamber receiving pulmonary veins and a distal compartment containing left atrial appendage. To our knowledge, this is the first report of cor triatriatum sinistrum associated with double outlet right ventricle.

Aberrant systemic artery-pulmonary venous fistula: diagnosis with Doppler imaging.

A 6-year-old asymptomatic girl presented with a continuous murmur at two different locations. Using Doppler imaging modalities, a small patent arterial duct and an aberrant systemic artery arising near the coeliac axis, piercing the right hemidiaphragm, and connecting to the right lower pulmonary vein were identified. Angiography confirmed the diagnosis and revealed additional pulmonary abnormalities. Doppler examination helped in planning appropriate angiographic projections and sites of the contrast medium injection.

Arteriovenous malformation of the vein of Galen in a newborn diagnosed with color Doppler examination--a case report.

A fourteen-day-old newborn infant presented with severe heart failure due to arteriovenous malformation of the vein of Galen. It was diagnosed by two-dimensional, pulsed-wave and color Doppler echocardiographic imaging. The latter method showed the afferent vessel to be the anterior cerebral artery entering an aneurysm of the vein of Galen at its posterior aspect. Information for surgical anatomic definition appears to be adequately provided by color Doppler examination, which permits avoidance of preoperative angiography.

Primary nephrotic syndrome in Qatar (Arabian Gulf).

Sixty-two children with primary nephrotic syndrome were studied. The mean age at diagnosis was 5.5 years (range 1.5-11 years). Fifty-five were initially steroid sensitive (88.7 per cent) and seven (11.2 per cent) were steroid resistant. Renal biopsies performed on six of the steroid-resistant cases showed focal glomerulosclerosis in three, minimal change disease in two and membranoproliferative glomerulonephritis in one. Of the 55 children who were initially steroid responsive, 12 (21.8 per cent) had a frequently relapsing course, while three (5.5 per cent) became steroid resistant. Two out of the 12 who had frequent relapses became steroid resistant and one became steroid dependent. Renal biopsies performed on six of these patients showed minimal change disease in five and segmental glomerulosclerosis in one. Renal biopsies of two out of three patients who were initially steroid responsive but who later became resistant showed minimal change disease in one and segmental sclerosis in one.