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.