OBJECTIVE Type 2 diabetes mellitus (T2DM) is associated with worsened clinical outcomes in hypertrophic cardiomyopathy (HCM) patients. We sought to investigate whether HCM patients with T2DM comorbidity exhibit adverse cardiac alterations in myocardial energetics, function, perfusion, or tissue characteristics. RESEARCH DESIGN AND METHODS A total of 55 participants with concomitant HCM and T2DM (HCM-DM) (n = 20) or isolated HCM (n = 20) and healthy volunteers (HV) (n = 15) underwent 31P-MRS and cardiovascular MRI. The HCM groups were matched for HCM phenotype. RESULTS Mean ± SD European Society of Cardiology sudden cardiac death risk scores were comparable between the HCM groups (HCM 2.2 ± 1.5%, HCM-DM 1.9 ± 1.2%; P = not significant), and sarcomeric mutations were equally common. HCM-DM patients had the highest median NT-proBNP levels (HV 42 ng/L [interquartile range 35–66], HCM 298 ng/L [157–837], HCM-DM 726 ng/L [213–8,695]; P < 0.0001). Left ventricular (LV) ejection fraction, mass, and wall thickness were similar between the HCM groups. HCM-DM patients displayed a greater degree of fibrosis burden with higher scar percentage and lower global longitudinal strain compared with HCM patients. PCr/ATP (the relative concentrations of phosphocreatine and ATP) was significantly lower in the HCM-DM group than in both HCM and HV (HV 2.17 ± 0.49, HCM 1.93 ± 0.38, HCM-DM 1.54 ± 0.27; P = 0.002). In a similar pattern, stress myocardial blood flow was significantly lower in the HCM-DM group than in both HCM and HV (HV 2.06 ± 0.42 mL/min/g, HCM 1.74 ± 0.44 mL/min/g, HCM-DM 1.39 ± 0.42 mL/min/g; P = 0.002). CONCLUSIONS We show for the first time that HCM-DM patients display greater reductions in myocardial energetics, perfusion, and contractile function and higher myocardial scar burden and serum NT-proBNP levels compared with patients with isolated HCM despite similar LV mass and wall thickness and presence of sarcomeric mutations. These adverse phenotypic features may be important components of the adverse clinical manifestation attributable to a combined presence of HCM and T2DM.

中文翻译：

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共存糖尿病与肥厚型心肌病患者不良表型特征的存在相关

目的 2 型糖尿病 (T2DM) 与肥厚性心肌病 (HCM) 患者的临床结果恶化有关。我们试图调查患有 T2DM 合并症的 HCM 患者是否在心肌能量学、功能、灌注或组织特征方面表现出不良的心脏改变。研究设计和方法 共有 55 名同时患有 HCM 和 T2DM (HCM-DM) (n = 20) 或单纯 HCM (n = 20) 的参与者和健康志愿者 (HV) (n = 15) 接受了 31P-MRS 和心血管 MRI . HCM 组与 HCM 表型相匹配。结果 欧洲心脏病学会心源性猝死风险评分的平均值 ± SD 在 HCM 组之间具有可比性（HCM 2.2 ± 1.5%，HCM-DM 1.9 ± 1.2%；P = 不显着），肌节突变同样常见。HCM-DM 患者的 NT-proBNP 水平中位数最高（HV 42 ng/L [四分位间距 35-66]，HCM 298 ng/L [157-837]，HCM-DM 726 ng/L [213-8,695]； P＜0.0001）。HCM 组的左心室 (LV) 射血分数、质量和壁厚相似。与 HCM 患者相比，HCM-DM 患者表现出更大程度的纤维化负担、更高的疤痕百分比和更低的整体纵向应变。HCM-DM 组的 PCr/ATP（磷酸肌酸和 ATP 的相对浓度）显着低于 HCM 和 HV（HV 2.17 ± 0.49，HCM 1.93 ± 0.38，HCM-DM 1.54 ± 0.27；P = 0.002）。在类似的模式中，HCM-DM 组的应激心肌血流量显着低于 HCM 和 HV（HV 2.06 ± 0.42 mL/min/g，HCM 1.74 ± 0.44 mL/min/g，HCM-DM 1.39 ± 0.42 毫升/分钟/克；P = 0.002）。结论 我们首次表明，与单纯 HCM 患者相比，HCM-DM 患者的心肌能量、灌注和收缩功能降低更大，心肌瘢痕负荷和血清 NT-proBNP 水平更高，尽管 LV 质量、壁厚和存在相似肌节突变。这些不良表型特征可能是 HCM 和 T2DM 合并存在导致的不良临床表现的重要组成部分。