부산대학교 도서관

Introduction: While aortic dissection in women with Turner syndrome (TS) has been well reported, other vascular dissections, including pulmonary artery (PA) dissections, have not been noted. In 2007, national guidelines were published to improve the care of women with TS and included recommendations for routine echocardiographic screening.Hypothesis: We sought to determine the incidence of vascular dissections in women with TS cared for since publication of the new guidelines and to assess for clinical correlates of such.Methods: Multi-centered retrospective data review of all cardiac, echocardiographic, endocrine, medicine and inpatient records was performed in patients with TS seen for clinical evaluation by any provider since 2007 at 3 institutions.Results: Of 603 patients identified, 395 were > 18 years of age at last visit. Within this cohort of adults, 75 (19%) were never referred to a cardiologist during their lifetime. At least 1 echocardiogram was performed in all but 27 (7%) patients. Since 2007, 12 (3%) women experienced a vascular dissection including 1 cerebral, 2 pulmonary artery and 8 aortic (1 root, 4 ascending, 1 arch, 1 left subclavian artery and 1 descending) of which 66% were fatal. Women with vascular dissections were older (42.5±16.0 years vs. 30.6 ±11.6 years; p=.007), more likely to have a bicuspid aortic valve (83% vs. 38%; p<0.001), prior repair of aortic coarctation (59% vs. 17%, p<0.001), pulmonary hypertension (25 vs. 10%, p=.01) and were less likely to have seen a cardiologist in the past 5 years (10% vs. 40%, p<0.001). There was no difference in BMI (31.3±9.7 vs. 29.0± 7.8, p=0.4), or presence of systemic hypertension (30% vs. 30%, p=0.96).Conclusions: Aortic dissection remains not uncommon in women with TS despite the 2007 Guidelines which call for routine periodic imaging. In addition to dissection of the ascending aorta, patients are at risk for dissection in other vessels. Pulmonary hypertension and lapse of cardiac care are newly identified modifiable risk factors which, if optimized may improve survival. Optimization of screening with routine periodic echocardiograms, in the absence of cardiac consultation, does not prevent dissection.