成人完全型肺静脉异位引流的超声诊断及术后评价

Echocardiographic diagnosis and post-operative evaluation of isolate total anomalous pulmonary venous connection in adults

目的:回顾性分析成人完全型肺静脉异位引流(TAPVC)术前和术后超声特征,提高临床对成人TAPVC的诊断和预后认识。方法:回顾性收集2011年1月至2019年9月,于北京安贞医院手术的16例成人TAPVC患者超声心动图,CT及相关临床资料,总结分析其超声特点及术后早期心脏结构及功能改变。结果:术前经超声心动图诊断,并经外科手术证实为TAPVC患者共16例,平均年龄(29.92±9.51)岁,临床症状有胸闷、体力活动不耐受及发绀。超声分型:心上型8例,心内型5例,混合型3例。15例(93.8%)术前超声分型与术中诊断一致。所有病例均存在非限制性房间隔交通(25.07±6.34)mm,其中继发肺动脉高压15例,合并肺动脉狭窄1例,所有病例均无肺静脉回流受阻。16例手术均成功,无严重并发症和死亡,术后早期右心室内径恢复正常[(34.92±6.61)vs.(20.23±3.79)mm,P<0.05],肺动脉高压患者肺动脉压力显著下降[(70.50±23.64)vs.(32.7±12.56)mmHg(1 mmHg=0.133 kPa),P<0.05],血氧饱和度逐渐趋向正常。结论:超声心动图是诊断成人TAPVC的首选方法,成人TAPVC常因合并非限制性房间隔交通或右心室流出口梗阻而与婴儿患者有不同的血流动力学特点及临床预后,外科手术效果良好。

Objective:We retrospectively analyzed the echocardiographic characteristics in adult patients with total anomalous pulmonary venous connection(TAPVC)before and after operation as to improve the awareness of diagnosis and prognosis.Methods:We retrospectively collected echocardiography,computed tomography and clinical data of 16 adult TAPVC patients in our center from January 2011 to December 2019.The echocardiographic characteristics,early postoperative cardiac structure and function changes were analyzed.Results:A total of 16 patients were diagnosed with isolate TAPVC by echocardiography and were confirmed by surgery,with an average age at operation of(29.92±9.51)years.Clinical symptoms included chest tightness,physical activity intolerance and cyanosis.Echocardiography showed 8 cases with supracardiac type,5 cases with intracardiac type,and 3 cases of mixed type.Echocardiographic classification in 93.8%(15/16)of cases were consistent with the surgical result.All 16 patients had nonrestrictive atrial septal defect(25.07±6.34)mm,15 patients with secondary pulmonary arterial hypertension(PAH),1 case had concurrent pulmonary stenosis(PS).There was no pulmonary vein drainage obstruction in all cases.Successful operations were completed in all without serious complications and death.After surgery,the right ventricle diameter markly reduced[(34.92±6.61)vs.(20.23±3.79)mm,P<0.05],also pulmonary arterial systolic pressure significantly de-creased[(70.50±23.64)vs.(32.7±12.56)mmHg,P<0.05]in PAH patients.Oxygen saturation tended to be normal.Conclusions:Echo is the preferred modality for diagnosing TAPVC.TAPVC in adult patients often has different hemodynamic characteristics and prognosis compared with infant patients due to nonrestrictive interatrial communication or right ventricular outflow obstruction.The postoperative outcome and early recovery in these patients was satisfactory.