住院确诊心肌致密化不全101例患者临床特点及超声心动图特征和预后分析

目的观察心肌致密化不全（NVM）患者的临床特点、超声心动图特征和预后。方法对2010--2012年在北京安贞医院住院确诊的101例NVM患者的临床症状、超声心动图、预后进行统计，随访主要不良事件包括心律失常、血栓栓塞、心功能恶化。分析临床特点和超声心动图数据对终点事件（死亡和心脏移植）的预测价值。结果101例NVM患者中67例（66．3％）男性，34例（33．7％）女性，年龄0～79岁，平均（29±25）岁。11例患者（10．9％）〉160岁，其中≥70岁的患者有4例（4．0％）。出现症状至确诊年限2．12（0．53～6．98）年，无症状者8例（8．0％），气促69例（68．3％），胸痛19例（18．8％），晕厥4例（4．0％），脑血管事件1例（1．0％），纽约心脏病学会（NYHA）心功能分级Ⅰ级14例（13．9％）、Ⅱ级37例（36．6％）、Ⅲ级31例（30．7％）、Ⅳ级19例（18．8％）。3例患者（3．0％）有心脏病家族史，4例患者（4．0％）至少有一名亲属有类似病变。57例患者（56．4％）出现充血性心力衰竭，9例患者（8．9％）合并扩张性心肌病。47例患者的首发症状是心力衰竭；14例首发症状是心律失常。56．4％的患者（57／101）左心室射血分数（LVEF）〈30％，22．8％的患者（23／101）LVEF≥50％，随访（2．6±1．4）年。严重并发症有：重度心力衰竭27例（26．7％）、心脏移植6例（6．0％），室性心律失常16例（15．8％），血栓事件3例（3．0％），死亡33例（32．7％）。死亡和心脏移植的相关因素有心功能不全NYHAHI级或Ⅳ级（风险比=3．98，P=0．04）、LVEF减低（风险比=0．89，P=0．006）、因心力衰竭加重住院（风险比=13．29，P〈0．001）。结论①心力衰竭患者在行超声心动图检查时初次发现NVM；②超声心动图检查经常诊断过度；③确诊NVM患者发生心脏移植、死亡不良事件风险大，需要严密随访。

The clinical features and prognosis of 101 cases with non-compaction of ventricular myocardium

Objective To describe the clinical, echocardiographic and prognostic features of non-compaction of ventricular myoeardium （NVM）. Methods Between 2010 and 2012, 101 suspected cases of NVM were i- dentified in Beijing Anzhen Hospital. The diagnosis of NVM was confirmed in 101 cases by echoeardiographic eval- uation in Anzhen Hospital echocardiography department. The main adverse events were arrhythmias, thromboembolism and heart function. Death or heart transplantation was the main endpoint of our study. Results The patients included 67 men （66.3%） and 34 women （33.7%） , aged 0-79 （29 ± 25 ） years. 11 （ 10.9% ） patients were 60 years or more at the time of echocardiography and 4 cases（4.0% ） were more than 70 years. There were 8 cases（8.0% ） ,shortness of breath 69 cases（68.3% ） , 19 chest pain cases（ 18.8% ） ,4 syncope cases（4.0% ）, cerebrovascular event 1 case（ 1.0% ） , New York heart association（NYHA） class Ⅰ 14 cases（ 13.9% ） ; class Ⅱ 37 eases（36.6% ） ; class Ⅲ 31 cases（30.7% ） ; class Ⅳ 19 cases（ 18.8% ）. A family history of cardiac disease was present in 3 cases （ 3.0% ）. Congestive heart failure was in 57 patients （ 56.4% ）, dilated cardiomyopathy was in 9 cases（8.9% ）. Left NVM was detected with heart failure symptoms in 47 patients and rhythm disorders in 14 patients. Left ventricular ejection fraction （LVEF） was 〈 30% in 56.4% and LVEF~〉50% was in 22.8%. During （ 2.6 ± 1.4） years of follow-up, several complications occurred, including severe heart failure in 27 cases （26.7%）, heart transplantation in 6 cases （6.0%）, ventrieular arrhythmia in 16 cases （ 15.8% ）, embolic e- vents in 3 cases（3.0% ） , and death in 33 cases （32.7%）. Factors that associated with death or heart transplan- tation were NYHA HI or IVhazard ratio（HR） =3.98, P=0.04], LVEF decreased （HR =0. 89, P =0.006） and hospitalization for heart failure （ HR = 13.29, P 〈 0. 001 ）. Conclusion （1）NVM is detected by screening echocardiography in heart failure patients. （2）NVM is frequently over-diagnosed by eehocardiography. （3）Patients i- dentified as NVM have a high risk of severe complications, transplantation or death.