中文摘要：

目的研究孤立性左室心肌致密化不全（LVNC）的临床特征和磁共振成像（MRI）表现。方法利用心脏MRI检查，采用不同成像序列对患者进行扫描，依据9节段分析法分析受累节段范围、程度及心脏功能等。此外引入舒张期受累节段致密化心肌厚度／室间隔基底段厚度（C／VS）比值试图对诊断标准进行优化。结果31例患者被诊断为LVNC，男23例，女8例，平均年龄39．9±15．7（13～64）岁。23例患者表现为心慌气短，其中9例初诊为扩张型心肌病。29例（93．5％）患者存在心电图异常，心律失常19例（61．3％）。31例患者共279个节段被分析，其中心肌致密化不全累及93个节段，占33．3％。31例患者的左室侧壁中段皆受累，23例（74．2％）患者左室心尖受累，其他依次为前壁中段17例（54．8％）、下壁中段10例（32．3％）、侧壁基底段8例（25．4％）、前壁基底段3例（9．7％）和下壁基底段1例（3．2％），室间隔基底段未见受累。84％的患者2个或2个以上节段受累；2例患者合并右室心尖部受累。3例合并左室附壁血栓，其中1例发生脑栓塞。MRI测量左室舒张末期横径58．7±10．2（45～89）min，左室射血分数37．2％±16．5％（14％～70％）。舒张期受累节段非致密化心肌厚度／致密化心肌厚度（N／C）比值3．6±1．4（2．2～9．2）；C／VS比值0．43±0．11（0．27～0．69）。结论心脏MRJ能够全面而准确地诊断LVNC，C／VS比值的测量可能会部分弥补常规诊断标准（N／C）的不足。

英文摘要：

Objective To observe the clinical and magnetic resonance imaging （MRI） characterizations in patients with isolated left ventricular noncompaction （LVNC）. Methods All patients were examined by MRI. The LV was divided into 9 segments for localizing noncompacted segments. A new value, C/VS, was introduced to assess the degree of noncompacted segments. Results A total of 31 patients was diagnosed as LVNC （23 males; 39.9 ± 15.7 years）. Palpitations presented in 74% of patients, abnormal EKG found in 93.5% of patients, 33. 3% segments were affected and most commonly in the midventricular and apical segments, 84% of patients had ≥2 affected segments. Right ventricle was affected in 2 patients. Left ventricular thrombi were detected in 3 patients. LVEF was 37.2% ±16. 5% （ 14% -70% ）, N/Cwas3.6±1.4（2.2-9.2）andC/VS was 0.43±0.11 （0.27-0.69）.Conclusions Cardiac MRI allows accurate LVNC assessment.