【摘要】 目的 探讨成人左室心肌致密化不全（LVNC）的临床特征及不同亚型的分布情况，分析致密化不全程度与心脏结构及功能的相关性。方法 本研究为多中心、回顾性研究。纳入年龄≥18岁且符合Jenni标准和/或Petersen标准的患者，收集其基线临床资料。结果 共纳入244例患者，患者确诊的中位年龄为48岁（35～59岁），其中70.6%为男性。中位左室射血分数（LVEF）为46%(35%～64%)，其中LVEF<50%的患者131例（53.7%）。在163例完成心脏磁共振的患者中，中位致密层厚度为3.60 mm(2.95～5.00 mm)，中位非致密层厚度为12.50 mm(9.97～16.22 mm)，中位NC/C值为2.80(2.40～3.60)。最常见的心律失常为室性早搏，共86例（35.2%）。最常见的LVNC亚型为扩张型，共118例（48.4%）。致密化不全累及部位与左室舒张末内径无明显相关（rs=-0.078,P=0.337），与LVEF无明显相关（rs=0.177,P=0.028）；非致密层厚度/致密层厚度（NC/C）比值与左室舒末内径（LVEDD）无明显相关（rs=-0.04,P=0.645），与LVEF无明显相关（rs=-0.051,P=0.557）。结论 成人LVNC确诊的中位年龄为48岁，多数为男性，超过半数的患者LVEF降低；扩张型LVNC是最常见的临床亚型；而致密化不全程度与心脏的结构及功能无显著相关性。更多还原

【Abstract】 Objective We conducted a multicenter clinical observational study to comprehensively describe the clinical features of adult LVNC and the distribution of different subtypes and further analyze the correlation between the degree of noncompaction and cardiac structure and function. Methods This was a retrospective multicenter study. Patients with diagnosed or suspected LVNC between January 2010 and April 2022 from 4hospitals in northwestern China were enrolled. Patients aged≥18 years and who fulfilled the Jenni criteria by echocardiography or the Petersen criteria by cardiovascular magnetic resonance were included. Patients with incomplete baseline data were excluded. We collected their baseline clinical data, including demographic characteristics, comorbidities, clinical manifestations, treatment, imaging, and electrocardiographic features.Results A total of 244 patients were finally included. The median age of patients at diagnosis was 48 years(35～59years), and 70.6% were male. The median left ventricular ejection fraction(LVEF) was 46%(35%～64%). LVEF <50% was present in 131 patients(53.7%), and LV dilation was present in 154 patients(63.1%). Among the 163patients who completed cardiac magnetic resonance, the median, minimum end-diastolic compaction thickness was 3.60 mm(2.95～5.00 mm), the median maximum end-diastolic non-compaction thickness was 12.50 mm(9.97～16.22mm), and the median NC/C ratio was 2.80(2.40～3.60). One hundred twenty-six patients(51.6%) had at least one arrhythmia; premature ventricular contractions(35.2%) were the most common. The most common subtype of LVNC was dilated, with 118 patients(48.4%). Non-compaction parts were not significantly related to left ventricular enddiastolic diameter(LVEDD)(rs=-0.078, P=0.337) and LVEF(rs=0.177, P=0.028); NC/C ratio was not significantly related to LVEDD(rs=-0.04, P=0.645) and LVEF(rs=-0.051, P=0.557). Conclusions The median age at diagnosis of adult LVNC was 48 years, and most of them were male; LVEF decreased in more than half of the patients; dilated LVNC was the most common clinical subtype; and there was no significant correlation between the degree of noncompaction and cardiac structure and function.更多还原