Natural course of isolated pulmonary valve stenosis in infants and children utilizing Doppler echocardiography.

Most natural history data regarding pulmonary stenosis (PS) were obtained from cardiac catheterization studies over 15 to 20 years ago. Selection bias in these studies often excluded patients with mild disease and infants. Today, Doppler echocardiography allows accurate serial assessments of stenotic lesions in patients of all ages. This study evaluates the natural history of PS utilizing serial Doppler examinations in the pediatric population. A total of 147 patients with PS and serial echocardiographic data were identified. Age at initial echocardiogram ranged from 2 days to 15 years, with a mean follow-up of 2.4 years. Sixteen of 56 patients (29%) initially evaluated within 1 month had a > or = 20 mm Hg increase in their peak systolic pressure gradient. Only 7 of 89 patients (8%) initially evaluated over 1 month had a > or = 20 mm Hg increase. Eleven of 40 newborn infants (28%) with mild obstruction had progression to moderate or severe PS compared with 10 of 68 patients (15%) initially evaluated over 1 month. Moderate PS in the newborn was also more likely to progress compared with older children. Of the 16 newborns with > or = 20 mm Hg increases, 8 developed the increase in < or = 6 months. In contrast, no patient aged >2 years whose initial gradient was <50 mm Hg developed severe PS. Mild PS may not be static, particularly in young infants. Progression in this age group occurs more often and more rapidly than in older infants and children.

Pulmonary embolus in an adolescent on oral contraceptives.

Deep venous thrombosis with pulmonary embolus is a rare complication of oral contraceptives, which generally occurs in adult women and becomes more common with increasing age. These complications are believed to be less common with low dose oral contraceptives than with the higher dose forms. This is a case report of a deep venous thrombosis presenting with a life-threatening pulmonary embolus in an adolescent on low dose triphasic oral contraceptives.

PIP: A 16 year old teenager with chest pain came to the emergency room at Children's Hospital in Charleston, South Carolina. Her sharp, constant midsternal pain and breathing difficulties began the morning of admission. 1 week before admission, she experienced a similar but less intense pain which resolved spontaneously. She was taking low-dose, triphasic oral contraceptives (OCs) as treatment for severe dysmenorrhea and dysfunctional uterine bleeding. She had no family history of elevated lipids, blood or clotting disorders, and cardiovascular disease. She did not smoke. She was obese (137.3 kg). She had elevated blood pressure (147/33 mm Hg), a high low-density lipoprotein count (140 mg/dL), and a high total cholesterol count (237 mg/dL). Her tender right calf was larger than the left calf. She had decreased perfusion of the entire left lung and right lower lobe. A pulmonary angiogram revealed emboli, and ultrasound of the legs revealed a thrombus behind the right knee. The physicians discontinued the OCs. They administered 2 anticoagulants--intravenous heparin followed by oral sodium warfarin for 6 months. Her respiratory distress ended within several days. Her right calf was no longer tender after 1 week. The physicians discharged her on a low-cholesterol, low-fat diet. They suggested she reduce her weight and exercise more often. She did not experience another venous thrombosis or pulmonary embolus. It appeared that the OCs contributed to her thromboembolism; yet, she did lead a sedentary lifestyle and was considerably overweight. Even though thromboembolism is rare in adolescents with no underlying predisposition for cardiovascular disease and who do not smoke, physicians should watch for sizeable changes in blood pressure, lipids, and cholesterol level as well as symptoms of deep venous thrombosis and cardiovascular disease.

Cardiomyopathy with arrhythmias and ectodermal dysplasia: a previously unreported association.

Hidrotic ectodermal dysplasia represents a group of congenital or hereditary disorders that involve ectodermal derivatives. It is characterized by partial or complete alopecia, dystrophic nails, and dental abnormalities. Dilated cardiomyopathy has not previously been reported in association with this illness. We report the cases of three children with fatal dilated cardiomyopathy with associated cardiac arrhythmias and ectodermal dysplasia. Laboratory investigations revealed no specific cause for the cardiomyopathy. It is speculated that this association is not simply coincidental.