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Abstract：
BACKGROUND: The interventricular septum has an important role in bi-ventricular performance. We hypothesized that septal involvement in apical hypertrophic cardiomyopathy (ApHCM-Mixed) adversely impacts ventricular structure and function when compared with isolated apical hypertrophy (ApHCM-Pure).
METHODS: A total of 72 patients (ApHCM-Mixed = 36, ApHCM-Pure = 36) with serial 2D and speckle-tracking echocardiographic analyses were identified. Ventricular function and mechanics were characterized by left (LV) and right (RV) ventricular global longitudinal strain (GLS), RV free wall strain, and LV myocardial work indices, and clinical events were adjudicated.
RESULTS: Clinical characteristics were similar between groups (mean age, 66 ± 15 years; 49% female; LV ejection fraction, 68 ± 11%). The ApHCM-Mixed group had larger LV mass indexes (141 ± 39 vs. 111 ± 30 g/m2, p < 0.001), worse LV (-9.6 ± 3.1 vs. -14.4 ± 3.4%, p < 0.001) and RV GLS (-14.3 ± 6.7 vs. -19.2 ± 5.2%, p = 0.001), impaired RV free wall strain (-18.5 ± 7.4 vs. -22.4 ± 6.3%, p = 0.02), and lower LV myocardial work indices including global work index (938 ± 306 vs. 1272 ± 339 mmHg%, p < 0.001), when compared with the ApHCM-Pure group. At a mean follow-up of 3.9 years, these differences all persisted. Five deaths were observed, all occurring in the ApHCM-Mixed group (14% vs. 0, p = 0.05), and with four being cardiac-related. This subgroup had a mean LV ejection fraction of 63%, LV GLS of -8.7%, an LV global work index of 875 mmHg%, and RV free wall strain of -15.9%, indicating significant subclinical bi-ventricular dysfunction.
CONCLUSIONS: ApHCM-Mixed represents a distinct morphology in hypertrophic cardiomyopathy associated with more impaired ventricular function and mechanics when compared with ApHCM-Pure.
METHODS: A total of 72 patients (ApHCM-Mixed = 36, ApHCM-Pure = 36) with serial 2D and speckle-tracking echocardiographic analyses were identified. Ventricular function and mechanics were characterized by left (LV) and right (RV) ventricular global longitudinal strain (GLS), RV free wall strain, and LV myocardial work indices, and clinical events were adjudicated.
RESULTS: Clinical characteristics were similar between groups (mean age, 66 ± 15 years; 49% female; LV ejection fraction, 68 ± 11%). The ApHCM-Mixed group had larger LV mass indexes (141 ± 39 vs. 111 ± 30 g/m2, p < 0.001), worse LV (-9.6 ± 3.1 vs. -14.4 ± 3.4%, p < 0.001) and RV GLS (-14.3 ± 6.7 vs. -19.2 ± 5.2%, p = 0.001), impaired RV free wall strain (-18.5 ± 7.4 vs. -22.4 ± 6.3%, p = 0.02), and lower LV myocardial work indices including global work index (938 ± 306 vs. 1272 ± 339 mmHg%, p < 0.001), when compared with the ApHCM-Pure group. At a mean follow-up of 3.9 years, these differences all persisted. Five deaths were observed, all occurring in the ApHCM-Mixed group (14% vs. 0, p = 0.05), and with four being cardiac-related. This subgroup had a mean LV ejection fraction of 63%, LV GLS of -8.7%, an LV global work index of 875 mmHg%, and RV free wall strain of -15.9%, indicating significant subclinical bi-ventricular dysfunction.
CONCLUSIONS: ApHCM-Mixed represents a distinct morphology in hypertrophic cardiomyopathy associated with more impaired ventricular function and mechanics when compared with ApHCM-Pure.
摘要：
背景：室间隔在双心室表现中具有重要作用。我们假设与孤立的心尖肥大（ApHCM-Pure）相比，心尖肥厚型心肌病（ApHCM-Mixed）的间隔受累会对心室结构和功能产生不利影响。
方法：共有72例患者（ApHCM-Mixed=36，ApHCM-Pure=36）进行了连续2D和斑点追踪超声心动图分析。心室功能和力学特征为左（LV）和右（RV）心室整体纵向应变（GLS），RV自由壁应变，和左心室心肌工作指数，并判定临床事件.
结果：组间临床特征相似（平均年龄，66±15岁；女性49%；左心室射血分数，68±11%)。ApHCM-Mixed组左心室质量指数较大（141±39vs.111±30g/m2，p<0.001），更差的LV(-9.6±3.1vs.-14.4±3.4%，p<0.001）和RVGLS（-14.3±6.7vs.-19.2±5.2%，p=0.001),受损RV自由壁应变(-18.5±7.4vs.-22.4±6.3%，p=0.02),和较低的左心室心肌工作指数，包括全球工作指数(938±306vs.1272±339mmHg%,p<0.001)，与ApHCM-Pure组相比。平均随访3.9年，这些差异都持续存在。观察到5人死亡，所有发生在ApHCM混合组中(14%与0，p=0.05），其中四个与心脏有关。这个亚组的平均左心室射血分数为63%，LVGLS为-8.7%，LV全球工作指数为875mmHg%，RV自由壁应变为-15.9%，表明显著的亚临床双心室功能障碍。
结论：与ApHCM-Pure相比，ApHCM-Mixed在肥厚型心肌病中表现出独特的形态，与更多的心室功能和力学受损有关。
方法：共有72例患者（ApHCM-Mixed=36，ApHCM-Pure=36）进行了连续2D和斑点追踪超声心动图分析。心室功能和力学特征为左（LV）和右（RV）心室整体纵向应变（GLS），RV自由壁应变，和左心室心肌工作指数，并判定临床事件.
结果：组间临床特征相似（平均年龄，66±15岁；女性49%；左心室射血分数，68±11%)。ApHCM-Mixed组左心室质量指数较大（141±39vs.111±30g/m2，p<0.001），更差的LV(-9.6±3.1vs.-14.4±3.4%，p<0.001）和RVGLS（-14.3±6.7vs.-19.2±5.2%，p=0.001),受损RV自由壁应变(-18.5±7.4vs.-22.4±6.3%，p=0.02),和较低的左心室心肌工作指数，包括全球工作指数(938±306vs.1272±339mmHg%,p<0.001)，与ApHCM-Pure组相比。平均随访3.9年，这些差异都持续存在。观察到5人死亡，所有发生在ApHCM混合组中(14%与0，p=0.05），其中四个与心脏有关。这个亚组的平均左心室射血分数为63%，LVGLS为-8.7%，LV全球工作指数为875mmHg%，RV自由壁应变为-15.9%，表明显著的亚临床双心室功能障碍。
结论：与ApHCM-Pure相比，ApHCM-Mixed在肥厚型心肌病中表现出独特的形态，与更多的心室功能和力学受损有关。
