A retrospective analysis was undertaken to define the natural history and long-term follow-up of a group of patients with Marfan's syndrome. Eighty-four patients were diagnosed between January 1959 and June 1987 as having Marfan's syndrome; 68% were male; their ages ranged from 2 to 67 years (mean 26.6). Sixteen patients constituted the early surgical group (those who underwent surgery before 1979; mean age 36.1 years). Nineteen patients constituted the late surgical group (surgery in 1979 or later; mean age 33.3 years). The nonsurgical group comprised 49 patients (mean age 19.3 years). Fifty-seven percent of the patients had a diastolic murmur and 38% had cardiomegaly at presentation. Fifty-seven percent underwent cardiac catheterization, which revealed aortic root dilation (85%), aortic regurgitation (73%), aortic dissection (33%) and mitral regurgitation (36%). Thirteen of the 19 patients in the late surgical group received a composite graft repair of the ascending aorta as compared with only 2 of the 16 in the early surgical group. Follow-up information was obtained on 81 (96%) of 84 patients; the follow-up time was 2 to 332 months (mean 99). Thirty-one of the 81 patients died at age 3 to 63 years (mean age 35 years); 87% of the known causes of death were related to the cardiovascular system. Sixty-one percent of deaths were the result of aortic dissection or rupture or sudden cardiac death. Of the 50 survivors, 98%, including all patients in the late surgical group, were in functional class I or II. Overall survival at 5, 10 and 15 years after operation was 78.4%, 57.1% and 49.5%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

CT-guided cryoablation is a safe, effective, reproducible procedural option for the nonoperative local treatment of painful musculoskeletal metastatic disease.

To compare four recently proposed methods of analyzing the exercise electrocardiogram with the conventional analysis of ST segment depression, 303 consecutive patients without myocardial infarction who had been referred for coronary arteriography underwent stress electrocardiography and stress thallium imaging. The specificity for the prediction of a greater than 50% coronary obstruction of 0.5, 1.0, 1.5 and 2.0 mm ST segment depression measured in the conventional way was 0.59, 0.73, 0.88 and 0.94, respectively. The specificity of a thallium perfusion defect was 0.79. Sensitivities of the conventional ST depressions, thallium defect, the change in the sum of the R amplitudes and the slope adjusted for heart rate increase were calculated and compared at the cited levels of specificity. R wave changes had a significantly lower sensitivity than did the conventionally analyzed ST depression at each level of specificity. Slope-adjusted ST depression had a slightly higher sensitivity than that of conventional ST depression only at a specificity of 0.73 (0.68 versus 0.65, p = 0.07). R wave-adjusted ST depression was significantly more sensitive than conventional ST depression only at a specificity of 0.94 (0.45 versus 0.36, p = 0.01). Heart rate-adjusted ST depression was more sensitive than conventional ST depression at all of the specificities except 0.59. This pattern of superior accuracy of heart rate-adjusted ST depressions was preserved for the prediction of multivessel coronary disease. Heart rate adjustment is a simpler and more accurate modification of the conventional electrocardiographic analysis than are the other three methods studied.

No abstract

A 3-year-old boy, who presented with progressive cyanosis and hypoxia, was diagnosed with a large congenital extrahepatic portosystemic shunt, interrupted IVC with azygos continuation, and multiple congenital anomalies. Traditionally open and laparoscopic surgical techniques have been used to treat this malformation. Endovascular repair using a 16-mm Amplatzer vascular plug (AGA Medical Corporation, Golden Valley, Minnesota, USA) was used to occlude the shunt. Immediate post-placement venography demonstrated cessation of flow within the shunt and increased portal venous flow. The patient's hypoxia and cyanosis decreased significantly, and he was discharged on the 5th post-procedure day in stable clinical condition. Three months follow-up evaluation demonstrated the vascular plug in place, unchanged in position.