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Coarctation resection in children with Turner's syndrome: a note of caution
Insights
Patients with Turner's syndrome undergoing aortic coarctation repair face significantly higher surgical risks, including aortic rupture and death. Careful surgical planning and precautions are crucial for this patient subgroup.
Area of Science:
- Cardiology
- Pediatric Surgery
- Genetics
Background:
- Coarctation of the aorta is a congenital heart defect requiring surgical intervention.
- Turner's syndrome is a genetic condition affecting females, associated with various health issues.
- The surgical risks for coarctation of the aorta in patients with Turner's syndrome are not well-defined.
Observation:
- Eight children with Turner's syndrome were identified among 353 patients undergoing coarctation of the aorta repair.
- Three patients with Turner's syndrome experienced perioperative hemorrhage due to aortic rupture, leading to one death and paraparesis.
- Two other patients with Turner's syndrome required angioplasty due to aortic wall friability, unlike patients without Turner's syndrome.
Findings:
- Patients with Turner's syndrome had a significantly higher rate of perioperative complications, including aortic rupture and death (p < 0.001).
- Aortic wall friability was noted in patients with Turner's syndrome, influencing surgical approach.
- No perioperative aortic rupture or neurologic deficits occurred in the control group without Turner's syndrome.
Implications:
- Surgical treatment for coarctation of the aorta in Turner's syndrome patients carries substantially increased operative risk.
- Special precautions are recommended, including specific surgical instruments and meticulous blood pressure management.
- Individualized surgical indications and techniques are essential for managing coarctation of the aorta in patients with Turner's syndrome.
Abstract:
Eight children were recognized to have Turner's syndrome, among 353 patients over 1 year of age who had undergone surgical treatment for coarctation of the aorta. Of these eight children, three developed a significant perioperative hemorrhage from aortic rupture, resulting in one death and one instance of paraparesis related to a period of prolonged hypotension. In two of the other five patients with Turner's syndrome, a decision was made to perform an angioplasty rather than a resection of the coarctation because of apparent friability of the aortic wall. In contrast, only one of the 345 patients without Turner's syndrome died as a result of surgical treatment, and none developed spontaneous perioperative aortic rupture or neurologic deficit. This experience suggests that the operative risk for coarctation of the aorta in this subgroup of patients is considerably 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 as well as the type of operative procedure must be individualized cautiously in patients with Turner's syndrome.