Study of prenatal diagnosis and classification and prognostic evaluation in fetuses with pulmonary atresia with intact ventricular septum / 中华超声影像学杂志

ABSTRACT

To explore the prenatal diagnosis classification and prognostic evaluation of fetal pulmonary atresia with intact ventricular septum ( PA/IVS) . Methods Thirty‐nine fetal PA/IVS were classified by the developmental condition of the right ventricle and ventriculo‐coronary artery communication ( VCAC) ,and tricuspid Z score was calculated . The associated abnormality ,chromosome abnormality were follow‐up analyzed . Results Fifteen fetuses were diagnosed with type Ⅰ PA/IVS ,14 fetuses with type ⅡPA/IVS ,and 10 with type Ⅲ PA/IVS . One case with type Ⅰ was associated with right aortic arch ,and other 38 fetuses were not associated with other cardiac abnormalities . T hirty‐nine fetuses were normal karyotype .Fetuses with type Ⅰ PA/IVS manifested right ventricular inlet portion ,well‐developed trabecular and infundibulum portions ,and no VCAC . T he tricuspid Z score of type Ⅰ PA/IVS was from －0 .07 to －2 .82 ,and 9 of the fetuses had biventricular repair and 6 had termination . Type Ⅱ PA/IVS manifested right ventricular trabecular portion absence ,small inlet and infundibulum portions ,and no VCAC . T he tricuspid Z score of type Ⅱ PA/IVS was from －3 .35 to －5 .21 ,and 7 of the fetuses had single ventricle palliation ,2 underwent fetal interventional procedures ,and 5 had termination . Type Ⅲ PA/IVS manifested absence of right ventricular trabecular and infundibulum portions ,small inlet portion ,and VCAC . T he tricuspid Z score of type Ⅲ PA/IVS was from －4 .33 to －6 .01 ,and 4 of the fetuses had single ventricle palliation and 6 had termination . The area under the ROC curve of tricuspid Z score in diagnosing PA/IVS postnatal biventricular repair was 1 .000 ( P ＜0 .01 ,95% CI1 .00－1 .00) ,the cutoff value was －3 .08 ,the sensitivity was 100% ,and the specificity was 100% . Conclusions Echocardiography can perform diagnostic classification of fetal PA/IVS and obtain fetal tricuspid valve Z score of ＞ －3 .08 and predict the postnatal outcome in PA/IVS . T he findings may have important implication for prenatal diagnosis and prognosis evaluation for PA/IVS .