Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, MA 02111, USA.
Circ Cardiovasc Imaging. 2012 Jul;5(4):441-7. doi: 10.1161/CIRCIMAGING.112.972760. Epub 2012 May 4.
In hypertrophic cardiomyopathy (HCM), cardiovascular MR can detect morphological abnormalities of the left ventricle (LV) not visualized with echocardiography. Although myocardial crypts (ie, narrow, blood-filled invaginations within the LV wall) have been recognized in HCM, all clinical implications of these structural abnormalities within the broad clinical HCM spectrum are not completely resolved. Therefore, we sought to characterize the prevalence and diagnostic significance of myocardial crypts in HCM patients.
Cine and late gadolinium enhancement cardiovascular MR and 2-dimensional echocardiography were obtained in 292 consecutive patients with HCM including 31 genotype-positive/phenotype-negative family members without LV hypertrophy (28 ± 16 years; 51% male) and 261 patients with LV hypertrophy (46 ± 18 years; 60% male). Ninety-eight subjects without cardiovascular disease were controls. Myocardial crypts (1-6/patient) were identified only by cardiovascular MR in 19 of 31 genotype-positive/phenotype-negative patients (61%) compared with only 10 of 261 (4%) patients with HCM with LV hypertrophy (P<0.001) and were absent in control subjects. Twelve-lead electrocardiograms were normal in 10 (53%) of the genotype-positive/phenotype-negative patients with crypts. Crypts were confined to the basal LV, most commonly in the ventricular septum (n=21) or posterior LV free wall (n=4), and associated with normal LV contractility and absence of late gadolinium enhancement in all but one patient.
LV myocardial crypts represent a distinctive morphological expression of HCM, occurring with different frequency in HCM patients with or without LV hypertrophy. Crypts are a novel cardiovascular MR imaging marker, which may identify individual HCM family members who should also be considered for diagnostic genetic testing. These data support an expanded role for cardiovascular MR in early evaluation of HCM families.
在肥厚型心肌病（HCM）中，心血管磁共振（cardiovascular MR）可以检测到超声心动图无法显示的左心室（LV）形态异常。尽管在 HCM 中已经认识到心肌隐窝（即 LV 壁内狭窄的、充满血液的内陷），但这些结构异常在广泛的 HCM 谱中的所有临床意义尚未完全解决。因此，我们试图描述 HCM 患者心肌隐窝的发生率和诊断意义。
对 292 例连续 HCM 患者进行了电影和晚期钆增强心血管磁共振和二维超声心动图检查，包括 31 名基因型阳性/表型阴性的无 LV 肥厚的家族成员（28±16 岁；51%为男性）和 261 名 LV 肥厚的患者（46±18 岁；60%为男性）。98 名无心血管疾病的受试者为对照组。心肌隐窝（1-6/患者）仅通过心血管磁共振在 31 名基因型阳性/表型阴性患者中的 19 名（61%）中被识别，而在 261 名伴有 LV 肥厚的 HCM 患者中仅 10 名（4%）（P<0.001），且在对照组中不存在。有隐窝的 31 名基因型阳性/表型阴性患者中，12 导联心电图正常者 10 名（53%）。隐窝局限于 LV 基底段，最常见于室间隔（n=21）或 LV 后游离壁（n=4），除 1 名患者外，所有患者均伴有正常 LV 收缩功能和无晚期钆增强。
LV 心肌隐窝是 HCM 的一种独特形态学表现，在伴有或不伴有 LV 肥厚的 HCM 患者中的发生率不同。隐窝是一种新的心血管磁共振成像标志物，它可能识别出也应考虑进行诊断性基因检测的 HCM 家族成员。这些数据支持心血管磁共振在 HCM 家族的早期评估中发挥更广泛的作用。
