Lastinger Lauren, Lee Marc, Hassen Lauren, Cavus Omer, Rajpal Saurabh, Moore Jeremy P, Mah May Ling, Bradley Elisa A
The Ohio State University, Department of Internal Medicine, Division of Cardiovascular Medicine, Columbus, OH, USA.
The Heart Center, Nationwide Children's Hospital, Columbus, OH, USA.
Int J Cardiol Congenit Heart Dis. 2024 Mar;15. doi: 10.1016/j.ijcchd.2024.100500. Epub 2024 Feb 17.
Sudden cardiac death (SCD) is an important risk for adults with repaired coarctation of the aorta (rCoA). We aimed determine if there are clinical risk factors for SCD in adults with rCoA.
SCD events and clinical data from all adults with rCoA at a tertiary care center (2007-2017) were evaluated. In 167 adults with rCoA (39 ± 11 years old, 75 (45%) female) SCD occurred in 8 (5%) (vs. age-matched adults 0.9%). Those with SCD demonstrated significant QTc prolongation (QTc: 479 ± 16 vs. 434 ± 30 msec, p < 0.001). Overall, adults with rCoA and a prolonged sex-normative QTc interval had a 12-fold increased risk of SCD (x (1) = 12.3, p < 0.001), with men sustaining SCD at younger ages (42 ± 13 years vs. women 60 ± 10 years, p < 0.05). Multiple logistic regression modeling demonstrated that prolonged QTc selectively advanced risk for SCD in men only (x QTc prolongation 8.46, p < 0.005 and x age 0.29, p = 0.587), whereas in women, age was associated with SCD risk (x QTc prolongation 2.84, p = 0.092 and x age 7.81, p = 0.005). Non-sustained ventricular tachycardia, ventricular dysfunction, and myocardial fibrosis did not significantly impact SCD risk.
There is an unanticipated high burden of SCD in adults with rCoA, occurring in men at younger age than women, suspicious for primary electrophysiologic dysfunction. Future investigation of sex-specific SCD risk in rCoA is important to better understand this disease and its late phenotype.
心脏性猝死（SCD）是主动脉缩窄修复术后（rCoA）成人患者的一项重要风险。我们旨在确定rCoA成人患者发生SCD的临床危险因素。
对一家三级医疗中心所有rCoA成人患者（2007 - 2017年）的SCD事件和临床数据进行评估。167例rCoA成人患者（年龄39±11岁，75例（45%）为女性）中，8例（5%）发生SCD（与年龄匹配的成人患者相比为0.9%）。发生SCD的患者表现出显著的QTc延长（QTc：479±16 vs. 434±30毫秒，p<0.001）。总体而言，rCoA且性别标准化QTc间期延长的成人患者发生SCD的风险增加12倍（χ(1)=12.3，p<0.001），男性发生SCD的年龄更小（42±13岁 vs. 女性60±10岁，p<0.05）。多因素逻辑回归模型显示，QTc延长仅选择性地增加男性发生SCD的风险（QTc延长χ=8.46，p<0.005，年龄χ=0.29，p=0.587），而在女性中，年龄与SCD风险相关（QTc延长χ=2.84，p=0.092，年龄χ=7.81，p=0.005）。非持续性室性心动过速、心室功能障碍和心肌纤维化对SCD风险无显著影响。
rCoA成人患者中SCD负担出乎意料地高，男性发生年龄比女性小，怀疑存在原发性电生理功能障碍。未来对rCoA中性别特异性SCD风险的研究对于更好地理解这种疾病及其晚期表型很重要。
