Department of Cardiology, VU University Medical Center, De Boelelaan 1117, Amsterdam, The Netherlands.
Eur Heart J Cardiovasc Imaging. 2012 Apr;13(4):292-7. doi: 10.1093/ehjci/jes005. Epub 2012 Jan 24.
Crypts can be found with cardiovascular magnetic resonance imaging (CMR) in hypertrophic cardiomyopathy (HCM) mutation carriers without hypertrophy (carriers) using a modified two-chamber view through the inferoseptum, but also in other patients and healthy individuals with standard long-axis views. Since it is currently unknown if carriers display a specific crypt morphology, we compared crypts in carriers with other cardiac pathologies (controls). Besides, we aimed to determine the optimal imaging plane for the detection of crypts by comparing modified two-chamber views with standard long-axis views. Finally, we evaluated the accuracy of crypts to identify carriers in HCM family screening.
Standard CMR long-axis views with additional modified two-chamber views were prospectively performed in carriers (n= 43), consecutive CMR control patients (n= 252), and mutation-negative family members (n= 15). Crypts were found in 70% (30/43) of carriers and in 12% (31/252) of controls (P< 0.001). Crypts in carriers showed deeper penetrance into the myocardium compared with controls (74 ± 21% vs. 59 ± 22%, P< 0.01). Detection of two or more crypts had a sensitivity of 51% and specificity of 94% for carriership. Modified two-chamber views doubled the sensitivity to detect crypts compared with standard long-axis views. In family screening, ≥2 crypts had a 100% positive predictive value to identify carriers.
Multiple crypts in the absence of left ventricular hypertrophy are highly specific for HCM mutation carriership and warrant clinical follow-up. A modified two-chamber view has a superior sensitivity compared with standard long-axis views for crypt detection. CMR may be of additional value to identify carriers in family screening.
在无心肌肥厚的肥厚型心肌病（HCM）突变携带者中，通过改良的下间隔双腔视图，在心血管磁共振成像（CMR）中可以发现隐窝，但在其他患者和有标准长轴视图的健康个体中也可以发现隐窝。由于目前尚不清楚携带者是否表现出特定的隐窝形态，我们比较了携带者与其他心脏病变（对照组）的隐窝。此外，我们旨在通过比较改良的双腔视图与标准长轴视图，确定检测隐窝的最佳成像平面。最后，我们评估了隐窝在 HCM 家族筛查中识别携带者的准确性。
前瞻性地对携带者（n=43）、连续的 CMR 对照组患者（n=252）和突变阴性的家族成员（n=15）进行标准 CMR 长轴视图加附加改良双腔视图。在 70%（30/43）的携带者和 12%（31/252）的对照组中发现了隐窝（P<0.001）。与对照组相比，携带者的隐窝更深地穿透心肌（74±21%比 59±22%，P<0.01）。检测到两个或更多隐窝对携带者的检测具有 51%的敏感性和 94%的特异性。与标准长轴视图相比，改良的双腔视图使隐窝的检测灵敏度提高了一倍。在家族筛查中，≥2 个隐窝对识别携带者具有 100%的阳性预测值。
在没有左心室肥厚的情况下，多个隐窝高度提示 HCM 突变携带者，需要进行临床随访。改良的双腔视图比标准长轴视图对隐窝检测具有更高的敏感性。CMR 可能对家族筛查中识别携带者具有附加价值。
