Aortic dilation, dissection, and rupture in patients with Turner syndrome |
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| Authors: | A E Lin B M Lippe M E Geffner A Gomes J F Lois C W Barton A Rosenthal W F Friedman |
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| Affiliation: | 1. Northwestern University Feinberg School of Medicine, Department of Surgery, Chicago, IL, USA;2. Northwestern University Feinberg School of Medicine, Department of Medical Social Sciences, Chicago, IL, USA;1. Department of Radiation Oncology, Baylor College of Medicine, Houston, TX;2. Department of Radiation Oncology, University of Utah, Salt Lake City, UT;1. Douglas Cohen Department of Paediatric Surgery, The Children''s Hospital at Westmead, Westmead, NSW, Australia;2. Sydney Medical School, The University of Sydney, NSW, Australia;3. Pacific Association of Pediatric Surgeons Member;1. Department of Surgery, Division of Surgical Oncology, Rush University Medical Center, Chicago, IL;2. Department of Surgery, Division of Transplantation, Rush University Medical Center, Chicago, IL;3. Department of Surgery, Cantonal Hospital Winterthur, Winterthur, Zurich, Switzerland;4. University of Zurich, Institute of Physiology, Zurich, Switzerland;5. Department of Radiology, Division of Nuclear Medicine, Rush University Medical Center, Chicago, IL |
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| Abstract: | We report two patients with Turner syndrome who had aortic dissection and rupture, one with prior repair of coarctation. We also note the high incidence (8.8%) of unrecognized aortic root dilation in a group of 57 patients with Turner syndrome whom we prospectively evaluated by echocardiography. Our analysis and review of previously reported cases suggests that multiple risk factors may exist for aortic dissection, including coarctation, bicuspid aortic valve, and systemic hypertension, but that these need not be present. Aortic root dilation may be an additional finding that suggests the patient with Turner syndrome is also at risk. When it is present, magnetic resonance imaging visualizes the entire aorta and allows quantification of the site and degree of dilation. In patients with dissection, the aorta often exhibits pathologic evidence of cystic medial necrosis similar to the finding in patients with Marfan syndrome. Therapeutic methods to decrease risk, such as those directed toward prevention of bacterial endocarditis, blood pressure control, and perhaps prophylactic beta blockade or surgical reconstruction, may need to be considered. Patients with Turner syndrome, their families, and the physicians who care for them should be aware of the significance of unexplained chest pain, dyspnea, or hypotension as potential manifestations of aortic dissection or rupture. |
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