J.Jpn. Surg. Soc.. 80(3): 261-267, 1979

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Original article

SURGICAL CONSIDERATIONS OF ATYPICAL COARCTAION OF THE AORTA DUE TO AORTITIS SYNDROME

This report is concerned with an analysis of our experience with 10 patients with atypical coarctation of the aorta due to aortitis syndrome. All patients were young female with clinical sings of severe hypertension in the upper half body. On admission, seven patients had clinical inflammatory signs such as increased ESR and positive CRP. The coaractated segments were located in the thoraco-abdominal aorta in 8 patients and distal portion of the descending thoracic aorta in 2. The associated cardiovascular anomalies were occlusion of aortic arch branches in 6 patients renal stenosis in 4, aortic insufficiency in 3, and mitral stenosis in 1. Surgical technique used for atypical coarctation of the aorta varied with location, nature and extent of the lesion and with the associated anomalies, namely thoraco-abdominal bypass in 6 patients, patch graft in 2 and miscellaneous for the associated anomalies in 2. One patients died of low output syndrome following aortic valve replacement.
Eight patients, who had the reconstructive surgery for the atypical coarctation, survived the operation and had the decreased pressure gradients across the coarctated segment.
These patients were followed up from 4 months to 14 years. During the follow up periods, two patients died three and a half years, and four years and two months respectively. One patient with associated renal stenosis had perisistent hypertension and another two patients, who had patch graft, had recurrent hypertension in the late postoperative period.
Our experiences indicate that atypical coarctation of the aorta due to aortitis syndrome can be treated satisfactorily by long thoraco-abdominal bypass.
If there were associated renal stenosis or aortic insufficiency, renal revascularization or aortic valve replacement is also necessary to normalize the blood pressure and to decrease the left ventricular strain.

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