Atypical Thoracoabdominal Aortic Coarctation

Summary

Atypical aortic coarctation accounts for approximately 2% of all coarctations. The pathological background is supposedly congenital, but the role of Takayasu or autoimmune aortitis has been emphasized in recent studies. During 1980–1999 we registered 23 patients (8 females, 15 males) with atypical aortic coarctation. The age ranged between 2 and 50 years (mean 22 years). Morphology varied from short stenosis to involvement of the entire thoracoabdominal aorta. Clinical manifestations were hypertension (n = 22), claudication (n = 4), and malabsorption (n = 2). Excessive hypertension led to cardiac failure in four patients. Epigastric bruit was a typical sign. Digital Subtraction angiography provided the diagnosis. Localization was thoracic in 5 cases, thoracoabdominal in 6, suprarenal in 11, and infrarenal in 1. Surgery included patchplasty in 17, aortoaortic bypass in 4 (with reimplantation of visceral and renal arteries in 2), splenorenal bypass in 2, and splenohepatorenal bypass in 1. There was no early morbidity. The follow-up period ranged between 3 and 20 years (mean 8 years). An aortoenteric fistula developed in one patient but healed but after removal of the infrarenal patch. Two patients required repeated surgery 8 and 4 years, respectively, after primary repair (following growth). All patients ultimately became normotensive. We concluded that atypical aortic coarctation may be found in the background in hypertensive children or in young adults. Surgical repair provides good results, but normal growth may necessitate repeated surgery.