Early spontaneous closure of large arterial ducts in two term neonates with ebstein anomaly after failed attempts of transcatheter closure

Abstract

Introduction

The unique features of neonatal Ebstein's anomaly (EA) are the presence of pulmonary atresia, arterial duct (AD) patency, and the variability of pulmonary vascular resistance. Promoting early ductal closure after ruling out or relieving anatomic pulmonary obstruction might improve neonatal survival. Transcatheter ductal closure is an interesting option but can be challenging in tiny neonates with large tortuous AD due to the limited available sizes of low-profile approved devices.

Objective

We report on 2 consecutive term newborns with EA and large AD in whom mechanical AD stimulus during failed attempts of transcatheter closure led after 2 days to definitive closure.

Methods

Two consecutive term neonates with EA were approached from the femoral vein for an attempted ductal closure. An 8 mm-AVPII was implanted in the AD (pulmonary end: 6.3 mm, length 8.9 mm) of the first 9-day-old patient (3 kg) and was removed before release for important leak and device instability. Delivery of 10 mm AVPII was laborious and procedure was aborted after important bleeding. A MVP-9Q was implanted in the AD (pulmonary end: 7.6 mm, length 16.1 mm) of the second 14-day-old patient (2.8 kg) and was removed before release after immediate migration to the pulmonary artery trunk. The procedure was aborted in the absence of a larger low-profile occlusion device in the armamentarium.

Results/Expected results

Patients were sent back to the ICU for surveillance and a re-discussion of the management strategy. Follow-up ultrasound showed a spontaneous and progressive diminution in the AD size until complete closure two days postoperative with good follow-up outcomes.

Conclusion/Perspectives

Transcatheter closure should be attempted in similar cases requiring early ductal closure. Surgical ligation can be postponed a couple of days after failed interventions when clinically possible. Further studies seem interesting to evaluate whether mechanical stimulation of the ductal wall can be a useful alternative to initiate spontaneous ductal closure in pre-term and term neonates.