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首页> 外文期刊>The American Journal of Cardiology >Cardiac Magnetic Resonance Imaging Features in Hypertrophic Cardiomyopathy Diagnosed at < 21 Years of Age
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Cardiac Magnetic Resonance Imaging Features in Hypertrophic Cardiomyopathy Diagnosed at < 21 Years of Age

机译：在<21岁时诊断出肥厚性心肌病的心脏磁共振成像特征

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Hypertrophic cardiomyopathy (HC) is the most common inherited cardiomyopathy, with varied timing of phenotypic and clinical presentation. Literature describing cardiac magnetic resonance (CMR) imaging and late gadolinium enhancement (LGE) in young patients with HC is limited. This study included patients diagnosed with HC at young age ( 1% LGE, respectively). Sudden cardiac death (SCD) risk was assessed in patients >16 years of age using the European SCD risk score. A composite outcome of New York Heart Association class III-IV symptoms, aborted SCD, heart transplantation, and all-cause mortality was assessed via KaplanMeier curves with log-rank analysis. Overall, 126 patients were included (78 male; 62%). Median age of diagnosis was 15 (12 to 18) years. LGE was present in 81 (64%) patients, although only 4 (3%) patients had LGE >15%. Median age at CMR imaging was 19 (15 to 23) years. Patients with LGE had greater wall thickness (25 +/- 8 mm vs 22 +/- 7 mm, p = 0.01). Median European SCD risk score was 4.7 (2.9 to 6.5). Median follow-up was 6.5 (2.5 to 13) years with 26 patients (21%) meeting the composite outcome. There were no significant differences in composite outcome since age of diagnosis when stratified by presence/ absence of LGE (p = 1.0). The presence of LGE in young HC patients was not an independent risk factor for cardiovascular morbidity and mortality. Wall thickness was greater in patients with LGE. There remains a need for further evaluation of this unique HC cohort. (C) 2020 Elsevier Inc. All rights reserved.
机译：肥厚性心肌病（HC）是最常见的遗传性心肌病，其表型和临床介绍的各种时间。文献描述了心脏磁共振（CMR）成像和HC患者的年轻钆增强（LGE）受限。该研究包括在年轻时诊断患有HC的患者（分别为1％的LGE）。使用欧洲SCD风险评分评估患者> 16岁的患者中突发的心脏死亡（SCD）风险。通过Kaplanmeier曲线评估纽约心脏关联III-IV类症状，中止SCD，心脏移植和全因死亡率的纽约心脏关联症状，中止的SCD，心脏移植和全因死亡率。总体而言，包括126名患者（78名雄性; 62％）。中位年龄的诊断年龄为15（12至18）岁。 LGE存在于81例（64％）患者中，虽然只有4名（3％）患者的患者的LGE> 15％。 CMR成像的中位年龄为19（15至23日）年。 LGE患者壁厚更大（25 +/- 8mm Vs 22 +/- 7 mm，P = 0.01）。中位数欧洲SCD风险得分为4.7（2.9至6.5）。中位随访时间为6.5（2.5至13）年，26名患者（21％）符合复合结果。自诊断年龄以来，在通过存在/不存在的诊断时没有显着差异（p = 1.0）。年轻的HC患者的LGE存在不是心血管发病率和死亡率的独立危险因素。 LGE患者壁厚更大。仍然需要进一步评估这种独特的HC队列。 （c）2020 Elsevier Inc.保留所有权利。

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