2019 年第 4 期 第 14 卷

成人孤立性心肌致密化不全的临床特征及预后分析

Clinical features and prognosis of isolated noncompaction of ventricular myocardium in adults

作者：王欣杨喜惠刘晓伟郝晓艳阮燕萍何怡华

英文作者：

单位：100029首都医科大学附属北京安贞医院胎儿心脏病母胎医学研究北京市重点实验室心血管疾病精准医学北京实验室（王欣、刘晓伟、郝晓艳、阮燕萍、何怡华）；830001乌鲁木齐市妇幼保健院超声科（杨喜惠）

英文单位：

关键词：心肌致密化不全；临床特征；预后

英文关键词：

• 摘要：

• 【摘要】目的    总结并分析孤立性心肌致密化不全（NVM）成人患者的临床特征及预后。方法    收集首都医科大学附属北京安贞医院病案系统中2007年12月至2017年12月确诊的57例孤立性NVM成人患者的临床资料进行回顾性分析。随访患者预后，并将其分为发生不良事件组及无不良事件组，比较2组临床特征及超声心动图指标。结果    57例患者中男性46例（80.7%），入院年龄为(42±16)岁，纽约心脏病协会（NYHA）心功能分级Ⅲ/Ⅳ级27例（47.4%），合并心律失常31例（54.4%），以室性心律失常（19例，33.3%）最多见，合并血栓事件8例（14.0%）。53例（93.0%）患者左心室舒张末期内径增大，39例（68.4%）入院时左心室射血分数＜50%；合并二尖瓣中重度反流30例（52.6%）；29例（50.9%）非致密心肌累及乳头肌水平。本研究随访时间为4（0，9）年，失访5例(8.8%)；11例患者发生不良事件（5例死亡，1例心脏移植术，1例心律转复除颤器植入术，4例起搏器植入术），不良事件发生率为21.2%。发生不良事件组（11例）合并心律失常、左心室舒张末期内径＞70 mm、右心室增大、右心房增大、中-大量心包积液比例均高于无不良事件组（41例），差异均有统计学意义（均P<0.05）。结论    孤立性NVM成人患者预后较差，男性居多，合并心律失常多见，血栓事件发生率较低。入院时左心室舒张末期内径增大、左心室射血分数减低、二尖瓣中重度反流及非致密心肌累及乳头肌水平常见，且患者合并心律失常、左心室舒张末期内径＞70 mm、右心增大、中-大量心包积液时预后不佳。

• 【Abstract】Objective    To summarize the clinical features and outcomes of adults with isolated noncompaction of ventricular myocardium (NVM). Methods    Clinical data of 57 patients diagnosed of isolated NVM in Beijing Anzhen Hospital, Capital Medical University between December 2007 and December 2017 were retrospectively reviewed. Long-term adverse events(ADE) were observed and the patients were divided into ADE group and ADE-free group. Clinical records and echocardiographic data were analyzed.  Results    Forty-six males（80.7%） were involved and the mean age of 57 patients was （42±16）years. Cardiac functional grading of New York Heart Association demonstrated grade Ⅲ/Ⅳ in 27 cases（47.7%）. Thirty-one patients（54.4%） presented arrhythmia and ventricular arrhythmia was the most common type(19 cases, 33.3%). Eight patients（14.0%） had thromboembolic events. Fifty-three patients （93.0%） presented enlarged left ventricular end-diastolic dimension and 39 patients(68.4%) presented left ventricular ejection fraction＜50% on admission. Moderate to severe mitral regurgitation occurred in 30 patients (52.6%). Noncompaction of papillary muscle was observed in 29 patients(50.9%). During 4(0,9)years of follow-up, 5 cases(8.8%) were lost; 11 patients(21.2%) had adverse events, including 5 cardiac death, 1 cardiac transplantation, 1 cardioverter defibrillator implantion and 4 pacemaker implantation. Rates of arrhythmia, left ventricular end-diastolic diameter＞70 mm, right ventricular enlargement, right atrial enlargement and moderate to severe pericardial effusion in ADE group（11 cases） were significantly higher than those in ADE-free group（41 cases）（all P<0.05）. Conclusions    Prognosis of adult patients with isolated NVM is poor; men are the majority; arrhythmia is common and thromboembolism is rare. NVM patients usually present enlarged left ventricular end-diastolic dimension, decreased left ventricular ejection fraction, moderate to severe mitral regurgitation and non-compact papillary muscle. Arrhythmia, left ventricular end-diastolic diameter＞70 mm, right heart enlargement and moderate to severe pericardial effusion are associated with poor prognosis.