Abstract 19087: Improved Survival After Percutaneous Stenting of the Atrial Septum in Human Fetal Left Atrial Hypertension
Hypoplastic left heart syndrome with a highly restrictive or intact atrial septum (HLHS-RAS) has a very high mortality. The associated fetal LA hypertension results in abnormal lung development with lymphangiectasia and pulmonary vein muscularization. We report our experience of ultrasound-guided stenting of the human fetal atrial septum.
Methods and Results. Retrospective review of fetuses with HLHS-RAS or a variant that underwent active perinatal management from 2000-January 2012. Ten fetuses were identified. Two fetuses became hydropic and died in utero (33, 29 weeks). Four fetuses required the urgent creation of an atrial communication immediately after birth but subsequently died (5-54 days), secondary to pulmonary hypertension. More recently, the atrial septum was stented in four fetuses at 28-36 weeks without maternal general anesthesia. Elevated left atrial pressure, massive dilatation of the pulmonary veins and MRI estimated pulmonary perfusion all improved after stenting. Two fetuses developed stent stenosis with recurrence of LA hypertension in utero for approximately four weeks and 1 day respectively. Three of four stented fetuses were born by vaginal delivery. Atrial septectomy and additional procedures were performed within 72 hours of birth. Intraoperative lung biopsy demonstrated muscularized pulmonary veins and lymphangiectasia in all four. Two died at 17 and 53 days of age from pulmonary hypertension and sepsis, respectively. The other two are still alive, a significant improvement in survival (p=0.03).
Conclusion. Fetal left atrial decompression by stenting may improve survival in HLHS-RAS.
- © 2012 by American Heart Association, Inc.