Hypertrophic Cardiomyopathy Program, NYU Langone Health, New York City, NY, USA.
Leon Charney Division of Cardiology, NYU Langone Health, New York City, NY, USA.
Am J Cardiol. 2020 Jun 1;125(11):1700-1709. doi: 10.1016/j.amjcard.2020.02.013. Epub 2020 Mar 5.
Clinical spectrum of hypertrophic cardiomyopathy (HC) has been expanded to include patients with mild or no thickening of the left ventricle (LV), who nevertheless have outflow tract obstruction at rest or after exercise, due to systolic anterior motion (SAM) and ventricular septal contact, with mitral valve elongation and papillary muscles anomalies. Apical ballooning mimicking a takotsubo syndrome (TS) wall motion pattern can occur in HC with mild septal thickening when latent obstruction becomes unrelenting. To define the prevalence of anatomic abnormalities characteristic of HC in patients diagnosed with TS, we analyzed echocardiograms of 44 unselected TS patients, age 67±12 years, 95% women including studies performed before the event (n = 11, median 515 days) and after recovery of left ventricular function (n = 33, median 92 days, interquartile range = 29 to 327) and compared the findings to 60 age and sexed matched controls. Analysis of echocardiograms was blinded to event timing, and patient vs. control status. During the ballooning event, 13 patients (30%) had SAM including 9 with LV outflow obstruction, peak gradients 71±40 mmHg, as well as: ventricular septal thickening (16 ± 4 mm), elongated anterior leaflets (30 ± 3mm), and increased mitral coaptation to posterior wall distance (17 ± 5 mm), consistent with diagnosis of the HC phenotype. Compared to 31 TS patients without SAM, study patients with SAM had longer anterior leaflets (30 ± 3 vs 26 ± 4 mm, p = 0.006), thicker septum (16 ± 4 vs 12 ± 3 mm), increased coaptation to posterior wall distance (17 ± 5 vs 14 ± 4 mm, p < 0.04) and reduced distance from coaptation to septum (19 ± 5 vs 27 ± 5, p < 0.001). In the 13 patients with SAM, morphologic characteristics of HC persisted after normalization of LV function. In conclusion, a subset of patients experiencing TS events demonstrates a constellation of morphologic abnormalities characteristic of HC that persist after recovery of LV wall motion. These findings suggest that dynamic outflow obstruction may cause apical ballooning in susceptible patients.
肥厚型心肌病（HC）的临床谱已扩展到包括那些左心室（LV）轻度或无增厚的患者，然而这些患者在休息或运动后仍存在流出道阻塞，这是由于收缩期前向运动（SAM）和室间隔接触导致的，同时伴有二尖瓣延长和乳头肌异常。在 LV 室壁运动呈心尖球囊样改变的情况下，当潜在梗阻变得持续不缓解时，可能会出现类似于 Takotsubo 综合征（TS）的壁运动模式。为了确定在诊断为 TS 的患者中，存在肥厚型心肌病特征性解剖异常的比例，我们分析了 44 例未经选择的 TS 患者的超声心动图，年龄 67±12 岁，95%为女性，包括事件发生前（n = 11，中位数 515 天）和 LV 功能恢复后（n = 33，中位数 92 天，四分位距为 29 至 327）的研究，并将这些发现与 60 名年龄和性别匹配的对照组进行比较。超声心动图分析对事件时间和患者与对照组的情况进行了盲法。在球囊样变期间，13 例患者（30%）出现 SAM，包括 9 例 LV 流出道梗阻，峰值梯度 71±40mmHg，同时伴有室间隔增厚（16 ± 4mm）、前瓣延长（30 ± 3mm）和二尖瓣瓣叶后瓣缘距离增加（17 ± 5mm），符合肥厚型心肌病表型的诊断。与 31 例无 SAM 的 TS 患者相比，出现 SAM 的 TS 患者的前瓣更长（30 ± 3 与 26 ± 4mm，p = 0.006）、室间隔更厚（16 ± 4 与 12 ± 3mm）、瓣叶后瓣缘距离增加（17 ± 5 与 14 ± 4mm，p < 0.04）和瓣叶后瓣缘距离减少（19 ± 5 与 27 ± 5mm，p < 0.001）。在出现 SAM 的 13 例患者中，LV 功能恢复后仍存在肥厚型心肌病的形态学特征。结论：一组经历 TS 事件的患者表现出一组肥厚型心肌病的形态学异常，这些异常在 LV 壁运动恢复后仍然存在。这些发现提示，动态流出道梗阻可能导致易感患者出现心尖球囊样改变。
