COARCTATION RESECTION IN CHILDREN WITH TURNERS SYNDROME - A NOTE OF CAUTION

Abstract

Children (8) had Turner''s syndrome among 353 patients > 1 yr of age who underwent surgical treatment for coarctation of the aorta. Of these 8 children, 3 developed a significant perioperative hemorrhage from aortic rupture, resulting in 1 death and one instance of paraparesis related to a period of prolonged hypotension. In 2 of the other 5 patients with Turner''s syndrome, an angioplasty was performed rather than a resection of the coarctation because of apparent friability of the aortic wall. Of 345 patients without Turner''s syndrome 1 died as a result of surgical treatment and none developed spontaneous perioperative aortic rupture or neurologic deficit. The operative risk for coarctation of the aorta in this subgroup of patients is greater than that in patients without Turner''s syndrome (P < 0.001). Special precautions should include use of rubber-jaw vascular clamps, choice of technique to avoid tension at the anastomotic suture line and careful control of systemic blood pressure intraoperatively and postoperatively. Indications for surgical treatment of coarctation and the type of operative procedure must be individualized cautiously in patients with Turner''s syndrome.