The Journal of thoracic and cardiovascular surgery
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J. Thorac. Cardiovasc. Surg. · Feb 2019
Loeys-Dietz syndrome: Intermediate-term outcomes of medically and surgically managed patients.
Loeys-Dietz syndrome (LDS) is an aggressive connective tissue disorder associated with increased risk of aortic dissection and aneurysm rupture at an early age and smaller aortic diameters. We report our experience with LDS to better understand its natural history and treatment outcomes and help establish treatment guidelines. ⋯ Prophylactic aortic surgical outcomes in LDS are excellent. Surgical reintervention remains high, particularly after aortic dissections. Close surveillance of medically managed and postoperative patients and early prophylactic surgery are crucial to avoid aortic catastrophe and achieve a good long-term prognosis.