Juxtaductal aortic coarctation. Analysis of 84 patients studied hemodynamically, angiographically, and morphologically after age 1 year.
Although many studies of juxtaductal coarctation of the aorta have been reported, none has correlated clinical, hemodynamic, angiographic, anatomic, and operative findings. Of 84 patients (62 male and 22 female; age range, 1 to 49 years [mean 17]), all had murmurs; 76 had absent, diminished, or delayed femoral pulsations; 50 had cuff systolic blood pressures in the arm greater than 140 mm Hg, and 30 had diastolic pressures greater than 90 mm Hg. The average pressure gradients (mm Hg) by direct measurements above and below the coarctation in 35 patients were peak systolic, 45; mean, 17; and diastolic, 5. Rib notching, visible in chest roentgenograms in 43 patients, correlated directly with age and inversely with the diameter of the coarctation. Moderate or marked cardiomegaly by radiograph was present in only 1 of 48 patients with isolated coarctation and in 17 of 36 with associated cardiovascular malformations. Electrocardiograms were abnormal in more than two thirds of patients with associated anomalies, but were normal in more than three fourths of those with isolated coarctation. In 70 excised, serially sectioned coarctations the aortic lumens were completely occluded in 4 patients, up to 0.5 mm in internal diameter in 22 patients, from 0.6 to 2 mm in 26 patients, from 2.1 to 5 mm in 14, and greater than 5 mm in 4, and correlated directly with lumens measured angiographically. The most significant anatomic factor causing the coarctation was invagination of the media from the posterior aortic wall, but intimal proliferation (jet lesion) at and immediately distal to the invagination contributed to the narrowing. Three (each with associated anomalies) of 70 patients died early after coarctation repair. Systolic or diastolic blood pressures decreased early postoperatively in 58 (87%) of 67 surviving patients, and both pressures decreased in 42 (63%). Late postoperatively (mean follow-up, 4.7 years), the systolic blood pressure remained elevated in 25% of patients.
D000293:Adolescent; D000328:Adult; D001013:Aorta, Thoracic / Q000000981:diagnostic imaging; D001017:Aortic Coarctation / Q000473:pathology / Q000503:physiopathology* / Q000601:surgery; D006332:Cardiomegaly / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007223:Infant; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000503:physiopathology; D013902:Radiography, Thoracic; D013348:Subclavian Artery / Q000000981:diagnostic imaging; D013599:Systole