Ma Wei-Guo, Chou Alan S, Mok Salvior C M, Ziganshin Bulat A, Charilaou Paris, Zafar Mohammad A, Sieller Richard S, Tranquilli Maryann, Rizzo John A, Elefteriades John A
Aortic Institute at Yale-New Haven Hospital, Yale University School of Medicine, New Haven, CT, United States; Beijing Anzhen Hospital of Capital Medical University and Beijing Institute of Heart, Lung and Blood Vessel Diseases, Beijing, China.
Aortic Institute at Yale-New Haven Hospital, Yale University School of Medicine, New Haven, CT, United States.
Int J Cardiol. 2017 Aug 1;240:132-137. doi: 10.1016/j.ijcard.2017.04.080. Epub 2017 Apr 25.
Although family members of patients with aortic dissection (AoD) are believed to be at higher risk of AoD, the prognostic value of family history (FH) of aortic dissection (FHAD) in family members of patients with AoD has not been studied rigorously. We seek examine how much a positive FHAD increases the risk of developing new aortic dissection (AoD) among first-degree relatives.
Patients with AoD at our institution were analyzed for information of FHAD. Positive FHAD referred to that AoD occurred in index patient and one or more first-degree relatives. Negative FHAD was defined as the condition in which only one case of AoD (the index patient) occurred in the family. The age at AoD, exposure years in adulthood before AoD, and annual probability of AoD among first-degree relatives were compared between patients with negative and positive FHADs.
FHAD was positive in 32 and negative in 68 among the 100 AoD patients with detailed family history information. Mean age at dissection was 59.9±14.7years. Compared to negative FHAD, patients with positive FHAD dissected at significantly younger age (54.7±16.8 vs 62.4±13.0years, p=0.013), had more AoD events in first-degree relatives (2.3±0.6 vs 1.0±0.0, p<0.001), and shorter exposure years per AoD event (18.3±6.7 vs 43.1±8.5, p<0.001). Annual probability of AoD per first-degree relative was 2.77 times higher in patients with positive than negative FHADs (0.0100±0.0057 vs 0.0036±0.0014, p<0.001).
A positive FHAD confers a significantly increased risk of developing aortic dissection on family members, with a higher annual probability of aortic dissection, a shorter duration of "exposure time" before dissection occurs and a lower mean age at time of dissection.
虽然主动脉夹层（AoD）患者的家庭成员被认为患AoD的风险更高，但主动脉夹层家族史（FHAD）在AoD患者家庭成员中的预后价值尚未得到严格研究。我们试图研究阳性FHAD在一级亲属中增加新发主动脉夹层（AoD）风险的程度。
对我们机构的AoD患者进行FHAD信息分析。阳性FHAD是指索引患者及一名或多名一级亲属发生了AoD。阴性FHAD定义为家族中仅出现一例AoD（索引患者）的情况。比较了阴性和阳性FHAD患者的AoD发病年龄、成年后至AoD发病前的暴露年限以及一级亲属中AoD的年发病概率。
在100例有详细家族史信息的AoD患者中，32例FHAD为阳性，68例为阴性。夹层的平均年龄为59.9±14.7岁。与阴性FHAD相比，阳性FHAD患者的夹层发病年龄显著更小（54.7±16.8岁 vs 62.4±13.0岁，p = 0.013），一级亲属中的AoD事件更多（2.3±0.6次 vs 1.0±0.0次，p < 0.001），每次AoD事件的暴露年限更短（18.3±6.7年 vs 43.1±8.5年，p < 0.001）。阳性FHAD患者的一级亲属中AoD的年发病概率比阴性FHAD患者高2.77倍（0.0100±0.0057 vs 0.0036±0.0014，p < 0.001）。
阳性FHAD会使家庭成员患主动脉夹层的风险显著增加，主动脉夹层的年发病概率更高，夹层发生前的“暴露时间”更短，夹层发生时的平均年龄更低。
