Abstract:
Hypertrophic cardiomyopathy (HCM) is a genetic disorder in which left ventricular outflow tract obstruction critically affects symptoms and prognosis. Traditionally, left ventricular outflow tract obstruction was primarily attributed to septal hypertrophy with systolic anterior motion of the mitral valve. However, recent evidence highlights significant contributions from the mitral valve and papillary muscle anomalies, as well as an apical-basal muscle bundle observed in HCM patients. Accurate morphological assessment is essential when considering septal reduction therapy. While transesophageal echocardiography and cardiac magnetic resonance are recommended for assessing the anomalous structures, four-dimensional computed tomography offers superior spatial resolution and multiplanar reconstruction capabilities. These features enable the evaluation of details of the morphological anomalies, such as the apical-basal muscle band, papillary muscle anomalies, subaortic stenosis, and right ventricular outflow tract obstruction. Based on the detailed assessment of these morphological features, four-dimensional computed tomography has been utilized for planning of surgical correction in a comprehensive HCM center. This approach facilitates the intervention strategies and may improve outcomes in septal reduction therapy for obstructive HCM.
摘要:
肥厚型心肌病(HCM)是一种遗传性疾病,其中左心室流出道阻塞严重影响症状和预后。传统上,左心室流出道梗阻主要归因于室间隔肥大伴二尖瓣收缩前移。然而,最近的证据强调了二尖瓣和乳头状肌异常的重要贡献,以及在HCM患者中观察到的根尖-基底肌束。考虑中隔减少治疗时,准确的形态学评估至关重要。虽然建议使用经食管超声心动图和心脏磁共振来评估异常结构,四维计算机断层扫描提供了优越的空间分辨率和多平面重建能力。这些特征可以评估形态异常的细节,例如根尖-基底肌带,乳头状肌异常,主动脉瓣下狭窄,右心室流出道梗阻。根据对这些形态特征的详细评估,四维计算机断层扫描已被用于计划在一个全面的HCM中心的手术矫正。这种方法有助于干预策略,并可能改善阻塞性HCM中隔减少治疗的结果。