Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota.
Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota; Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota; Department of Molecular Pharmacology & Experimental Therapeutics, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, Rochester, Minnesota.
Am J Cardiol. 2020 Apr 15;125(8):1249-1255. doi: 10.1016/j.amjcard.2020.01.027. Epub 2020 Jan 28.
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 (<21 years) between January 1990 and January 2015 who underwent transthoracic echocardiography and CMR with assessment of LGE at a single tertiary referral center. LGE was quantified via a method of 6 standard deviations and patients were grouped based upon presence or absence of LGE (≤1% and >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 Kaplan-Meier 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.
肥厚型心肌病（HC）是最常见的遗传性心肌病，其表型和临床表现的时间各不相同。描述年轻 HC 患者心脏磁共振成像（CMR）和晚期钆增强（LGE）的文献有限。本研究纳入了 1990 年 1 月至 2015 年 1 月期间在一家三级转诊中心接受经胸超声心动图和 CMR 检查并评估 LGE 的年轻（<21 岁）HC 患者。通过 6 个标准差的方法对 LGE 进行量化，并根据是否存在 LGE（分别为≤1%和>1% LGE）对患者进行分组。>16 岁的患者使用欧洲 SCD 风险评分评估 SCD 风险。通过 Kaplan-Meier 曲线和对数秩分析评估纽约心脏协会心功能分级 III-IV 症状、心脏骤停、心脏移植和全因死亡率的复合终点。共有 126 例患者入选（78 例男性；62%）。中位诊断年龄为 15（12 至 18）岁。81 例（64%）患者存在 LGE，尽管仅有 4 例（3%）患者的 LGE>15%。CMR 成像的中位年龄为 19（15 至 23）岁。有 LGE 的患者壁厚度更大（25±8mm 比 22±7mm，p=0.01）。中位欧洲 SCD 风险评分为 4.7（2.9 至 6.5）。中位随访时间为 6.5（2.5 至 13）年，26 例（21%）患者达到复合终点。按 LGE 存在/不存在分层时，诊断年龄不同时复合终点无显著差异（p=1.0）。年轻 HC 患者中存在 LGE 不是心血管发病率和死亡率的独立危险因素。有 LGE 的患者壁厚度更大。仍需进一步评估这一独特的 HC 队列。
