Abstract: Background　Noncompaction of the ventricular  myocardium（NVM）of right ventricle is a relatively rare disease in clinic.Many patients may not have obvious clinical manifestations at the early stage.When patients come to the hospital with pulmonary hypertension or right heart failure，the possibility of diagnosing this disease is often ignored.Objective　To analyze the clinical manifestations and imaging features of patients with NVM of right ventricle，improving cliniciansl awareness of the disease.Methods　Clinical data of 10 patients with a definite diagnosis of NVM of right ventricle were recruited from General Hospital of Ningxia Medical University from January 2007 to February 2017.The demographic characteristics，clinical manifestations，electrocardiogram，echocardiography，other imaging findings，other test，outcomes were analyzed retrospectively.Results　 The 10 cases consisted of 4 females and 6 males，had an average age of（53.5±15.5） years（range：21-80）．4 of them had comorbidities，including 2 had hypertension，and 2 had hypertension and diabetes.The respiratory symptoms of these patients were dyspnea（6 cases），cough（3 cases），chest tightness and expectoration（2 cases），general edema and wheezing （1 case），and other discomforts（1 case）．The electrocardiogram showed atrial fibrillation（4 cases），right bundle branch block（2 cases），tachycardia with occasional premature ventricular contraction（2 cases），pulmonale（1 case），and normal results（2 cases）．Echocardiography showed that all of them had significantly enlarged right atrium and right ventricle，and NVM of right ventricle.Of the 10 patients，9 had thickened right ventricular anterior wall，8 had moderate to severe pulmonary hypertension，and 5 had pericardial effusion，2 had mild pulmonary hypertension.Left ventricular ejection fraction（LVEF） values ranged from 35% to 70% with mean LVEF values （57±13）%.Only 2 of the 10 cases had improved pulmonary ventilation perfusion imaging，and no significant embolization was found.CT pulmonary angiography（CTPA） was performed in 8 cases，and the results showed that all of them had significantly dilated pulmonary artery，6 were also found with bilateral pleural effusion in small amounts，4 were also found with pulmonary congestion.Pulmonary function tests in 8 patients showed normal ventilation function and airway resistance and small airway resistance disorder（6 cases），and normal lung function（2 cases）．Polysomnography in 6 patients indicated nocturnal hypoxemia.The mean nocturnal oxygen saturation（SaO2） was 84%.One of them had a minimum SaO2 of 64% at night.The mean AHI were 6.5 times/h.10 patients were followed up for 5 years，during which one died，but other cases were relieved.Conclusion　For those with unexplained pulmonary hypertension and right heart failure，clinicians should carefully consider whether the patients have  NVM of right ventricle.Early diagnosis and treatment of NVM of right ventricle help to prolong the survival and reduce the mortality of the patients.

Key words: Isolated noncompaction of the ventricular myocardium；Hypertension, pulmonary；Heart failure

摘要： 背景　右心室心肌致密化不全（NVM）是临床中比较少见的疾病，很多患者在早期可能没有明显的临床表现，当患者出现肺动脉高压或右心衰竭来院就诊时往往会忽略此病的可能。目的　分析右心室NVM患者的临床表现及影像学特征，提高临床上对该病的认识。方法　收集2007年1月—2017年2月宁夏医科大学总医院确诊的10例右心室NVM患者的临床资料，回顾性分析患者的一般资料、临床表现、心电图、心脏彩超、其他影像学检查、其他检查、转归情况等。结果　10例患者中女4例，男6例；年龄21~80岁，平均年龄（53.5±15.5）岁。其中4例患者有合并症，2例合并高血压，2例合并高血压和糖尿病。症状表现为6例有气促症状，3例有咳嗽症状，2例有胸闷、咳痰症状，1例有全身水肿、喘息症状，1例有其他不适症状。心电图表现为4例出现心房颤动，2例出现右束支传导阻滞，2例出现心动过速偶发室性期前收缩，1例出现肺型P波，2例心电图正常。10例患者心脏彩超均提示右心房、右心室显著增大，右心室NVM。10例患者中9例右心室前壁增厚，8例中重度肺动脉高压，5例心包积液，2例轻度肺动脉高压。左心室射血分数（LVEF）为35%~70%，平均LVEF（57±13）%。10例患者中有2例完善了肺通气灌注显像未见明显栓塞。8例患者螺旋CT肺动脉造影（CTPA）检查均可见肺动脉明显增宽，且其中6例双侧有少量胸腔积液、4例有肺淤血表现。8例患者肺功能检查显示6例常规通气功能及气道阻力正常，小气道阻力障碍，2例肺功能正常。6例患者多导睡眠监测报告结果均提示夜间低氧血症，平均夜间血氧饱和度（SaO2）为84%，其中1例患者夜间最低SaO2为64%，夜间监测呼吸暂停低通气指数（AHI）平均6.5次/h。10例患者随访5年，1例死亡，其他患者均缓解。结论　针对不明原因的肺动脉高压、右心衰竭的患者，临床医生应提高对右心室NVM所致可能的认识及警惕性，而早期的右心室NVM的诊断与治疗有助于延长患者的生存期，降低其病死率。

关键词: 孤立性心室肌致密化不全；高血压, 肺性；心力衰竭