Clair Mathieu, Fernandes Susan M, Khairy Paul, Graham Dionne A, Krieger Eric V, Opotowsky Alexander R, Singh Michael N, Colan Steven D, Meijboom Erik J, Landzberg Michael J
Department of Cardiology, Boston Children's Hospital, Boston, Mass, USA.
Congenit Heart Dis. 2014 May-Jun;9(3):235-43. doi: 10.1111/chd.12109. Epub 2013 Jun 14.
To determine the prevalence of aortic valve dysfunction, aortic dilation, and aortic valve and ascending aortic intervention in adults with coarctation of the aorta (CoA).
Aortic valve dysfunction and aortic dilation are rare among children and adolescents with CoA. With longer follow-up, adults may be more likely to have progressive disease.
We retrospectively reviewed all adults with CoA, repaired or unrepaired, seen at our center between 2004 and 2010.
Two hundred sixteen adults (56.0% male) with CoA were identified. Median age at last evaluation was 28.3 (range 18.0 to 75.3) years. Bicuspid aortic valve (BAV) was present in 65.7%. At last follow-up, 3.2% had moderate or severe aortic stenosis, and 3.7% had moderate or severe aortic regurgitation. Dilation of the aortic root or ascending aorta was present in 28.0% and 41.6% of patients, respectively. Moderate or severe aortic root or ascending aortic dilation (z-score > 4) was present in 8.2% and 13.7%, respectively. Patients with BAV were more likely to have moderate or severe ascending aortic dilation compared with those without BAV (19.5% vs. 0%; P < 0.001). Age was associated with ascending aortic dilation (P = 0.04). At most recent follow-up, 5.6% had undergone aortic valve intervention, and 3.2% had aortic root or ascending aortic replacement.
In adults with CoA, significant aortic valve dysfunction and interventions during early adulthood were uncommon. However, aortic dilation was prevalent, especially of the ascending aorta, in patients with BAV.
确定主动脉缩窄（CoA）成人患者中主动脉瓣功能障碍、主动脉扩张以及主动脉瓣和升主动脉干预的发生率。
主动脉瓣功能障碍和主动脉扩张在患有CoA的儿童和青少年中较为罕见。随着随访时间延长，成人患者可能更易出现疾病进展。
我们回顾性分析了2004年至2010年间在本中心就诊的所有CoA成人患者，无论是否接受过修复治疗。
共确定了216例CoA成人患者（男性占56.0%）。末次评估时的中位年龄为28.3岁（范围18.0至75.3岁）。65.7%的患者存在二叶式主动脉瓣（BAV）。在末次随访时，3.2%的患者有中度或重度主动脉狭窄，3.7%的患者有中度或重度主动脉反流。分别有28.0%和41.6%的患者出现主动脉根部或升主动脉扩张。分别有8.2%和13.7%的患者出现中度或重度主动脉根部或升主动脉扩张（z评分>4）。与无BAV的患者相比，有BAV的患者更易出现中度或重度升主动脉扩张（19.5%对0%；P<0.001）。年龄与升主动脉扩张相关（P=0.04）。在最近一次随访时，5.6%的患者接受了主动脉瓣干预，3.2%的患者进行了主动脉根部或升主动脉置换。
在CoA成人患者中早期成年期出现显著主动脉瓣功能障碍和干预并不常见。然而，BAV患者中主动脉扩张普遍存在，尤其是升主动脉。
