左房异构及其合并畸形的超声心动图诊断与治疗转归分析

目的分析左房异构及其合并畸形患者的超声心动图诊断特征及分型，并探讨其临床治疗及预后转归。 方法选取2010年1月至2018年10月于首都医科大学附属北京安贞医院行经胸超声心动图检查明确诊断为左房异构的患者47例。对其超声心动图诊断特征及分型、手术治疗方法及转归预后等进行回顾性分析。 结果47例左房异构患者中合并复杂畸形者35例（35/47，74.5%），其中肺血多型16例，肺血少型19例；合并简单畸形者10例（10/47，21.3%），无合并心内畸形者2例（2/47，4.2%）。47例左房异构患者中内脏位置异常2例；心脏位置异常14例，其中右位心11例（11/47，23.4%）、中位心3例（3/47，6.4%）；38例合并多脾畸形（38/47，80.9%），1例合并无脾畸形；42例合并支气管及肺脏畸形（42/47，89.4%）。47例均合并下腔静脉肝段缺如（47/47，100.0%）；合并上腔静脉连接异常者23例（23/47，48.9%）；合并肝静脉连接异常者47例（47/47，100.0%）；合并肺静脉异位引流6例（6/47，12.8%）。47例患者中9例（9/47，19.1%）存在心电图或动态心电图检查结果异常。47例患者中，26例接受矫治或姑息手术治疗，其中21例为复杂畸形，5例为简单畸形。接受手术治疗的26例患者中，4例死亡，均为合并复杂畸形患者。 结论左房异构患者多合并复杂心血管及其他脏器畸形，可通过超声心动图明确诊断。超声心动图对左房异构的确诊及合并畸形的准确评估，对临床治疗方案和目标的制定以及患者预后转归的判定具有重要意义。

Echocardiographic diagnosis and treatment of left atrial heterogeneity and associated malformations

ObjectiveTo analyze the echocardiographic diagnosis of left atrial isomerism, and to explore the clinical prognosis and prognosis of such patients. MethodsFrom January 2010 to October 2018, 47 cases of left atrial isomerism confirmed by echocardiography at Beijing Anzhen Hospital Affiliated to Capital Medical University were collected. The echocardiographic features, classification, surgical treatment, and prognosis were analyzed retrospectively. ResultsAmong 47 patients with left atrial isomerism, 35 (35/47, 74.5%) had complex malformations, 10 (10/47, 21.3%) had simple malformations, and 2 (2/47, 4.2%) were not complicated with malformations. Among the 35 cases with complex malformations, 16 were polytypic in lung blood and 19 were hypotypic. Among the 47 patients with left atrial isomerism, 2 had abnormal visceral position, and 14 had abnormal cardiac position, including 11 (11/47, 23.4%) cases of right heart and 3 (3/47, 6.4%) cases of middle heart; 38 were complicated with polysplenic malformation (38/47, 80.9%) and 1 complicated with asymptomatic malformation; 42 (42/47, 89.4%) were complicated with bronchial and pulmonary malformations; 47 were combined with absence of the hepatic segment of the inferior vena cava (47/47, 100.0%); 23 (23/47, 48.9%) had abnormal superior vena cava connection; 47 (47/47, 100.0%) had abnormal hepatic venous connection; 6 (6/47, 12.8%) had abnormal pulmonary venous drainage; 9 (9/47, 19.1%) had abnormal electrocardiogram or dynamic electrocardiogram findings; 26 underwent orthodontic or palliative surgery, of which 21 had complicated malformations and 5 had simple malformations. Among the 26 cases undergoing surgical treatment, 4 died, all of whom had complicated malformations. ConclusionLeft atrial isomerism patients are often complicated with cardiovascular and other organ malformations, which can be diagnosed by echocardiography. Echocardiography plays an important role in the diagnosis of left atrial isomerism and the accurate evaluation of associated malformations, as well as in the formulation of clinical treatment plans and the evaluation of the prognosis of patients.