Giugliano Gregory R, Sethi Prabhdeep S
Baystate Medical Center, 759 Chestnut Street, Cardiology S4659, Springfield, MA 01199, USA.
J Invasive Cardiol. 2009 Jun;21(6):e103-5.
Neurofibromatosis (NF) is a genetic disorder inherited in an autosomal dominant pattern. Subtypes I and II are the most well recognized, and among these, Type I has associated vasculopathy. Less than 3% of patients have vascular involvement, with the renal artery as the most commonly involved vessel. Dissection and rupture of aneurysms in larger arteries such as the subclavian and the aorta have been previously reported. This is the first reported case of a spontaneous coronary artery dissection in a patient with NF-I. Imperative in the percutaneous treatment of coronary artery dissections is early recognition and proactive decision making. Recognition of a possible association between NF and coronary dissections will further facilitate successful and prompt management of this otherwise rare entity.
神经纤维瘤病(NF)是一种以常染色体显性模式遗传的遗传性疾病。I型和II型是最广为人知的亚型,其中I型与血管病变有关。不到3%的患者有血管受累,肾动脉是最常受累的血管。此前已有锁骨下动脉和主动脉等较大动脉的动脉瘤夹层和破裂的报道。这是首例关于I型神经纤维瘤病患者发生自发性冠状动脉夹层的报道。冠状动脉夹层的经皮治疗中,关键在于早期识别和积极决策。认识到NF与冠状动脉夹层之间可能存在的关联,将进一步有助于成功且迅速地处理这种罕见病症。