Purnell Richard, Williams Ian, Von Oppell Ulrich, Wood Andrew
Department of Cardiology, University Hospital of Wales, Heath Park, Cardiff, UK.
Eur J Cardiothorac Surg. 2005 Aug;28(2):346-8. doi: 10.1016/j.ejcts.2005.05.004.
Noonan's syndrome is one of the commonest hereditary syndromes with cardiac involvement. We report a rare presentation of this syndrome, associated with giant aneurysms of the left and non-coronary sinuses of Valsalva, resulting in symptomatic aortic valve regurgitation. The patient's clinical investigations and operative management are presented. Histological examination of aortic tissues offers insight into the pathogenesis of these complications.
努南综合征是最常见的伴有心脏受累的遗传性综合征之一。我们报告了该综合征一种罕见的表现形式,与主动脉瓣窦(瓦尔萨尔瓦窦)的左窦和无冠状动脉窦的巨大动脉瘤相关,导致有症状的主动脉瓣反流。本文介绍了该患者的临床检查及手术治疗情况。主动脉组织的组织学检查有助于深入了解这些并发症的发病机制。