Division of Cardiology, Department of Internal Medicine, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, Changwon, Republic of Korea; Department of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Department of Cardiology, Rabin Medical Center, Faculty of Medicine, Tel Aviv University, Israel; Department of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Int J Cardiol. 2023 Jun 15;381:62-69. doi: 10.1016/j.ijcard.2023.04.006. Epub 2023 Apr 5.
Left ventricular (LV) morphologic progression in apical hypertrophic cardiomyopathy (AHC) has not been well studied. We evaluated serial echocardiographic changes in LV morphology.
Serial echocardiograms in AHC patients were assessed. LV morphology was categorized according to the presence of an apical pouch or aneurysm, and LV hypertrophic severity and extent; relative, pure, and apical-mid type defined as mild (<15 mm thickness) apical hypertrophy, significant (≥15 mm) apical hypertrophy, and both apical and midventricular hypertrophy, respectively. Adverse clinical events and late gadolinium enhancement (LGE) extent on cardiac magnetic resonance were evaluated for each morphologic type.
In 41 patients, 165 echocardiograms (maximal interval: 4.2 [IQR, 2.3-11.8] years) were evaluated. Morphologic changes were observed in 19 (46%) patients. Eleven (27%) patients displayed the progression of LV hypertrophy toward pure or apical-mid type. Five (12%) and 6 (15%) patients developed new pouches and aneurysms. Patients with progression tended to be younger (50 ± 15.6 vs 59 ± 14.4 years, P = 0.058) and had a longer period of follow-up (12 [5-14] vs 3 [2-4] years, P < 0.001). During a follow-up of 7.6 (IQR 3.0-12.1) years, 21 (51%) experienced clinical events. The relative, pure, and apical-mid types showed different LGE extents (2%, 6%, and 19%, P = 0.004). Patients with severe hypertrophic and apical involvement showed higher clinical event rates.
About half of AHC patients had a progression of LV morphology to more hypertrophic involvement and/or an apical pouch or aneurysm formation. Advanced AHC morphologic types were associated with higher event rates and scar burdens.
左心室（LV）在心尖肥厚型心肌病（AHC）中的形态学进展尚未得到充分研究。我们评估了 LV 形态的系列超声心动图变化。
评估 AHC 患者的系列超声心动图。根据是否存在心尖囊袋或瘤，以及 LV 肥厚的严重程度和范围，将 LV 形态分为心尖囊袋或瘤形成，以及 LV 肥厚程度和范围；根据轻度（<15mm 厚度）心尖肥厚、显著（≥15mm）心尖肥厚和心尖及中室肥厚分别定义为相对、单纯和心尖-中室型。评估每种形态类型的不良临床事件和心脏磁共振上的晚期钆增强（LGE）程度。
在 41 例患者中，共评估了 165 次超声心动图（最大间隔：4.2[IQR，2.3-11.8]年）。19 例（46%）患者观察到形态变化。11 例（27%）患者的 LV 肥厚向单纯或心尖-中室型进展。5 例（12%）和 6 例（15%）患者出现新的囊袋和瘤。进展的患者更年轻（50±15.6 岁比 59±14.4 岁，P=0.058），随访时间更长（12[5-14]年比 3[2-4]年，P<0.001）。在 7.6（IQR 3.0-12.1）年的随访中，21 例（51%）发生临床事件。相对、单纯和心尖-中室型的 LGE 程度不同（2%、6%和 19%，P=0.004）。严重肥厚和心尖受累的患者临床事件发生率更高。
约一半的 AHC 患者的 LV 形态向更严重的肥厚累及和/或心尖囊袋或瘤形成进展。晚期 AHC 形态类型与更高的事件发生率和瘢痕负荷相关。
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