分析先天性右心室双出口（DORV）新分型在胎儿先天性心脏病诊断中的应用价值。

回顾性分析中国医学科学院阜外医院2018年1月至2020年12月诊断为先天性DORV的胎儿的超声心动图资料，根据大动脉位置的关系、室间隔缺损与大动脉是否相关、肺动脉口是否狭窄进行DORV新分型，共分为Ⅰ~Ⅷ型。所有DORV胎儿均完成新分型诊断，并对其进行预后评估，分析总结DORV新分型诊断及随访预后结果。

共纳入43例先天性DORV胎儿，其中Ⅰ型4例（9.3%），Ⅱ型12例（27.9%），Ⅲ型5例（11.6%），Ⅳ型3例（7.0%），Ⅴ型8例（18.6%），Ⅵ型6例（14.0%），Ⅶ型2例（4.7%），Ⅷ型3例（7.0%）。经超声科、小儿心脏外科、产科给予预后咨询建议后，选择继续妊娠15例，出生率34.9%，其中Ⅰ型3例、Ⅱ型7例、Ⅲ型2例、Ⅳ型1例、Ⅴ型2例，出生后均复查心脏超声，主要诊断符合率100%，15例胎儿出生后均完成双心室矫治，预后良好。选择终止妊娠28例，原因包括：合并其他不可矫治的复杂畸形12例；室间隔缺损远离大动脉，无法实现双心室矫治10例；放弃治疗8例。43例DORV中21例产前评估预后良好，其中15例出生后成功进行手术治疗，救治率71.4%（15/21）。

DORV新分型可帮助获得精确的产前诊断和准确的预后评估，提高预后良好DORV的出生率和救治率。

To assess the diagnostic value of the new classification of double outlet right ventricle (DORV) in fetal congenital heart diseases.

Fetuses with DORV diagnosed by echocardiography at Fuwai Hospital, Beijing, China from January 2018 to December 2020 were enrolled in this retrospective study. Congenital DORV was categorized into eight types (Ⅰ-Ⅷ) according to three basic factors as follows: the positional relationships of great arteries (normal relation or abnormal relation), the relationships of the ventricular septal defect (VSD) to great arteries (committed VSD or remote VSD), and the presence or absence of pulmonary outflow tract obstruction. All the fetuses with DORV were diagnosed using the new classification, and integrated management was performed after prognosis consultation. Relevant diagnostic experience and follow-up results are summarized.

Forty-three congenital DORV fetuses were included in the study. Eight types of DORV were observed: type Ⅰ in four (9.3%) patients, type Ⅱ in twelve (27.9%), type Ⅲ in five (11.6%), type Ⅳ in three (7.0%), type V in eight (18.6%), type Ⅵ in six (7.0%), type Ⅶ in two (4.7%), and type Ⅷ in three (7.0%). After prognosis consultation by sonographers, pediatric cardiac surgeons, and obstetricians, continued gestation was recommended in 15 fetuses, with a birth rate of 34.9%, including three patients with type Ⅰ, seven with type Ⅱ, two with type Ⅲ, one with type Ⅳ, and two with type Ⅴ. Neonatal echocardiography was arranged after birth, and the accuracy of prenatal diagnosis was 100%. All of the patients had completed biventricular correction with a good prognosis. Twenty-eight patients chose induced labor, and the reasons included the combination of untreatable complex malformations in 12 cases, noncommitted ventricle septal defect (for which biventricular correction could not be completed) in 10, and abandoned treatment in 8. Twenty-one patients had a good prognosis, and the treatment rate was 71.4% (15/21).

The new classification of DORV could help provide detailed prenatal diagnosis and prognosis evaluation, which is beneficial to the birth rate and the treatment rate for fetuses with a good prognosis.