EP04.19: The estimation of the prognosis for fetal critical aortic stenosis with the left atrium area and the Doppler pattern in pulmonary veins

Abstract

Introduction: Fetal critical aortic stenosis (CAS) is a challenging lesion with poor prognosis such as fetal hydrops and intrauterine fetal death. It has been known that the double reverse pattern in pulmonary veins relates to poor prognosis. We had a case with CAS with fetal hydrops, and spontaneously resolved the hydrops with the increased forward flow in aorta. We evaluated the hemodynamic changes with pulmonary venous Doppler pattern as well as the left atrium area/ cardiac area ratio (LA/CA). Case: A 30 year old woman, gravida 2, para 1, was referred at 28 weeks of gestation for polyhydramnios, fetal hydrops and cardiac abnormality. Markedly enlarged left atrium and severe mitral regurgitation with CAS was detected. Aortic valve size was 1.9mm (z-score= -6.8). The LA/CA was 0.40 and the pulmonary venous Doppler showed double reverse pattern with forward/reverse velocity time integral (VTI) ratio (FRVR)= 1.18. Amnioreduction was performed due to polyhydramnios at 31 weeks gestation. After that, LA/CA decreased to 0.24, and the FRVR in pulmonary veins increased to 7.11. The forward flow through aorta started being seen spontaneously. The fetal hydrops was relieved with the reduction of the LA volume, but the left ventricular (LV) function remained poor. The male neonate weighing 2,171g was delivered by Caesarean section at 36 weeks of gestation with an Apgar score of 5 and 6 at 1 and 5 minutes, respectively. The neonate required the opening atrial septum defect and bilateral pulmonary artery banding right after birth. Norwood operation was planned owing to poor LV function. Discussion: Double reverse pattern in pulmonary veins might be reversible in the fetus with CAS. The change of FRVR in pulmonary veins and LA/CA would be helpful to understand the hemodynamic change in fetus with CAS. EP04.19: Table 1.