Study of prenatal diagnosis and classification and prognostic evaluation in fetuses with pulmonary atresia with intact ventricular septum

Abstract

Objective 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 Ⅱ PA/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. Thirty-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. The 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. The 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. The 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%CI: 1.00-1.00), 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. The findings may have important implication for prenatal diagnosis and prognosis evaluation for PA/IVS. Key words: Echocardiography; Fetus; Pulmonary atresia with intact ventricular septum; Prognosis; Tricuspid valve Z score