目的 评价经产前超声心动图诊断的完全性肺静脉异位连接（total anomalous pulmonary venous connection，TAPVC）胎儿围生期一体化管理的临床有效性。 方法 回顾性分析2017年1月—2021年12月在青岛市妇女儿童医院经产前超声心动图诊断的64例TAPVC胎儿围生期一体化管理的临床资料。围生期一体化管理是指对产前诊断为TAPVC的胎儿联合产科、胎儿医学科、超声科、小儿心脏外科、小儿麻醉科及新生儿科进行多学科联合诊治。 结果 64例TAPVC胎儿中，心上型29例，心内型27例，心下型2例，混合型6例。42例胎儿进行了染色体核型分析，均未见明显异常。64例TAPVC胎儿中，37例被引产，27例随访至足月出生。27例TAPVC患儿中，2例放弃治疗，2例转诊至外院治疗后失访，余23例进行一期根治手术。1例因低心排综合征于术后6个月内死亡，余22例随访（2.1±0.3）年，预后均良好（2例分别因吻合口狭窄、肺静脉狭窄在术后1年内接受了第2次手术）。 结论 TAPVC胎儿经围生期一体化管理多可获得良好预后。
Abstract
Objective To evaluate the clinical effectiveness of integrated management during the perinatal period for fetuses diagnosed with total anomalous pulmonary venous connection (TAPVC) by prenatal echocardiography. Methods Clinical data of 64 cases of TAPVC fetuses diagnosed by prenatal echocardiography and managed with integrated perinatal care in Qingdao Women and Children's Hospital from January 2017 to December 2021 were retrospectively analyzed. Integrated perinatal care included multidisciplinary collaboration among obstetrics, fetal medicine, ultrasound, pediatric cardiology, pediatric anesthesia, and neonatology. Results Among the 64 TAPVC fetuses, there were 29 cases of supracardiac type, 27 cases of intracardiac type, 2 cases of infracardiac type, and 6 cases of mixed type. Chromosomal analysis was performed in 42 cases, and no obvious abnormalities were found. Among the 64 TAPVC fetuses, 37 were induced labor, and 27 were followed up until term birth. Among the 27 TAPVC cases, 2 cases accepted palliative care, 2 cases were referred to another hospital for treatment and lost to follow-up, while the remaining 23 cases underwent primary repair surgery. One case died within 6 months after the operation due to low cardiac output syndrome, while the other 22 cases were followed up for (2.1±0.3) years with good outcomes (2 cases underwent a second surgery within 1 year after the first operation due to anastomotic stenosis or pulmonary vein stenosis). Conclusions TAPVC fetuses can achieve good outcomes with integrated management during the perinatal period.
关键词
完全性肺静脉异位连接 /
先天性心脏病 /
围生期 /
一体化管理 /
胎儿 /
儿童
Key words
Total anomalous pulmonary venous connection /
Congenital heart disease /
Perinatal period /
Integrated management /
Fetus /
Child
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