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K E Jefferson

Publications and source records attributed to K E Jefferson.

5 recordsLinked to original sources

Calcification of aortic homografts used for reconstruction of the right ventricular outflow tract.

Calcification of aortic homografts used for reconstruction of the right ventricular outflow tract was studied in assess the frequency of occurrence, location, extent, speed of progression, and clinical and hemodynamic implications. Radiologic follow-up by means of penetrated posteroanterior and lateral radiograms ranged from 2 to 10 years in two groups of patients. Group 1 comprised 40 patients with cyanotic congenital heart disease in whom an aortic homograft was used to restore continuity between the right ventricle and pulmonary arteries. Results from this group were correlated with those from Group 2, which included 80 patients who had a homograft implanted as a substitute for their own pulmonary valve, which had been used as an autograft for replacing the diseased aortic or mitral valve. In Group 1, 92% of the grafts were calcified 6 months to 4 years after operation. Calcification was severe in two cases, moderate in 33, and mild in two. One of the grafts had to be removed in the tenth postoperative year for progressive obstruction. In Group 2, 27% showed signs of calcification, never before the second year after implantation. Calcium development was severe in two, moderate in 12, and mild in eight. Two obstructed homografts were removed. Aortic homograft calcification has been more common, developed earlier, and been more severe in patients operated upon for congenital lesions. The lower age, the peculiar metabolism, the distorted anatomy, and the development of pulmonary hypertension in this group may account for this fact. In 96% of the patients who were followed, calcification has not yet meant clinical disability or hemodynamic dysfunction. Use of "fresh," antibiotic-sterilized homografts has reduced the incidence of calcification in Group 2 and is expected to decrease its severity in all cases.

Adolescent↗

Formes frustes of Marfan's syndrome presenting with severe aortic regurgitation. Clinicogenetic study of 18 families.

Eighteen patients who presented with severe aortic regurgitation and dilatation of the ascending aorta were found to be formes frustes of Marfan's syndrome and formed the basis for this clinicogenetic study. All had aortic valve replacement and reconstruction of part of the ascending aorta. The diagnosis was confirmed by histological examination of the aortic tissue. There were 126 first degree relatives; 85 were living and 67 (78-8%) of these were examined. Limited information was available about 32 of the 41 relatives who had died. No relative had the classical clinical features of Marfan's syndrome but stigmata of the disease were found in 25 (37-3%) of the 67 first degree relatives examined. In 21, the abnormality was confined to the cardiovascular system, the skeleton, or the eye, but in 4, abnormalities involved 2 systems. Cardiovascular abnormalities affecting the aortic valve or aortic wall were present in 6 (9-0%) of the 67 first degree relatives examined. One or more of the skeletal indices measured (height-span difference, metacarpal index, phalangeal index) was abnormal in 18 (26-9%) and ocular abnormalities were found in 5 of 51 (9-8%) examined. There were no relatives with dislocation of the lens or iridodonesis. Using strict diagnostic criteria, a minimum of 37-3 per cent of the first degree relatives examined were affected; this involved 12 of the 18 families studied. There was nothing in our data to suggest that the formes frustes of the disease had a different mode of inheritance from the classical syndrome.

Adult↗