Sherrid Mark V, Riedy Katherine, Rosenzweig Barry, Ahluwalia Monica, Arabadjian Milla, Saric Muhamed, Balaram Sandhya, Swistel Daniel G, Reynolds Harmony R, Kim Bette
Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York.
Mount Sinai St. Luke's, Icahn School of Medicine at Mount Sinai, New York City, New York.
Echocardiography. 2019 Jan;36(1):47-60. doi: 10.1111/echo.14212. Epub 2018 Dec 12.
Acute left ventricular (LV) apical ballooning with normal coronary angiography occurs rarely in obstructive hypertrophic cardiomyopathy (OHCM); it may be associated with severe hemodynamic instability.
METHODS, RESULTS: We searched for acute LV ballooning with apical hypokinesia/akinesia in databases of two HCM treatment programs. Diagnosis of OHCM was made by conventional criteria of LV hypertrophy in the absence of a clinical cause for hypertrophy and mitral-septal contact. Among 1519 patients, we observed acute LV ballooning in 13 (0.9%), associated with dynamic left ventricular outflow tract (LVOT) obstruction and high gradients, 92 ± 37 mm Hg, 10 female (77%), age 64 ± 7 years, LVEF 31.6 ± 10%. Septal hypertrophy was mild compared to that of the rest of our HCM cohort, 15 vs 20 mm (P < 0.00001). An elongated anterior mitral leaflet or anteriorly displaced papillary muscles occurred in 77%. Course was complicated by cardiogenic shock and heart failure in 5, and refractory heart failure in 1. High-dose beta-blockade was the mainstay of therapy. Three patients required urgent surgical relief of LVOT obstruction, 2 for refractory cardiogenic shock, and one for refractory heart failure. In the three patients, surgery immediately normalized refractory severe LV dysfunction, and immediately reversed cardiogenic shock and heart failure. All have normal LV systolic function at 45-month follow-up, and all have survived.
Acute LV apical ballooning, associated with high dynamic LVOT gradients, may punctuate the course of obstructive HCM. The syndrome is important to recognize on echocardiography because it may be associated with profound reversible LV decompensation.
在梗阻性肥厚型心肌病（OHCM）中，冠状动脉造影正常但出现急性左心室（LV）心尖部气球样变的情况很少见；它可能与严重的血流动力学不稳定有关。
方法、结果：我们在两个肥厚型心肌病治疗项目的数据库中搜索了伴有心尖部运动减弱/运动消失的急性左心室气球样变。OHCM的诊断依据是在没有导致肥厚和二尖瓣-室间隔接触的临床病因的情况下，按照左心室肥厚的传统标准进行。在1519例患者中，我们观察到13例（0.9%）出现急性左心室气球样变，伴有动态左心室流出道（LVOT）梗阻和高压力阶差，92±37mmHg，10例女性（77%），年龄64±7岁，左心室射血分数（LVEF）31.6±10%。与我们其余的肥厚型心肌病队列相比，室间隔肥厚较轻，分别为15mm和20mm（P<0.00001）。77%的患者出现二尖瓣前叶冗长或乳头肌向前移位。5例患者病程中并发心源性休克和心力衰竭，1例并发难治性心力衰竭。大剂量β受体阻滞剂是主要治疗方法。3例患者需要紧急手术解除LVOT梗阻，2例因难治性心源性休克，1例因难治性心力衰竭。在这3例患者中，手术立即使难治性严重左心室功能障碍恢复正常，并立即逆转了心源性休克和心力衰竭。在45个月的随访中，所有患者左心室收缩功能均正常，且全部存活。
与高动态LVOT压力阶差相关的急性左心室心尖部气球样变可能是梗阻性肥厚型心肌病病程中的一个特征。该综合征在超声心动图上很重要，因为它可能与严重的可逆性左心室失代偿有关。
