A Novel Hybrid Single Ventricular Assist Device (SVAD) Strategy for a Neonate with Ventricular Septal Defect, Aortic Arch Hypoplasia, and Biventricular Dysfunction

Abstract

Introduction Neonates requiring biventricular VAD (BiVAD) support remain a high-risk population. Common challenges include limited space for multiple cannulae, poor wound healing, and difficulties establishing adequate VAD fill and ejection. Given these concerns, we present a unique case using a hybrid procedure and SVAD implant in a neonate with two-ventricle(2V) circulation. Case Report Female term neonate (birth weight 3.2 kg; BSA 0.2 kg/m2) born with a moderate-sized ventricular septal defect (VSD), aortic arch hypoplasia, and severe biventricular dysfunction due to a MHY7 mutation. In light of her weight, likely need for biventricular support given her underlying cardiomyopathy and need for extensive arch repair, we elected to proceed with a hybrid SVAD support strategy. We hypothesized that this approach would decrease the duration of bypass and minimize the number of cannula over the traditional approach of VSD and arch repair with BiVAD support. We also hoped this strategy would provide improved LV decompression, thus improving pulmonary mechanics and nutritional support. On day 6 of life, she underwent atrial septectomy, bilateral PA band placement, and implant of a 10 ml Berlin EXCOR using a 6 mm EXCOR cannula in the right atrium and a 5 mm EXCOR cannula in the main pulmonary artery. A PDA stent was placed using a perventricular approach. She progressed well post-operatively with discontinuation of inotropic support on post-operative day (POD) 3 and extubation on POD 4. VAD fill and ejection have been appropriate. The clinical course is noteworthy for diaphragm paralysis requiring plication and ongoing BiPAP support. Additionally, she developed NEC on POD 23 and has only tolerated ∼30% of her nutritional needs enterally. To date, she has remained on VAD support > 100 days while awaiting transplant. Summary We present a unique case of a neonate with 2V congenital heart disease who has been supported with a hybrid SVAD strategy using a Berlin EXCOR. While this approach has been advantageous in avoidance of VSD and arch repair and requiring fewer cannulae, it also has had challenges; namely, the need for ongoing respiratory and feeding support. We speculate that this may reflect the challenges of optimal PA band placement as well as difficulties providing balanced systemic and pulmonary flow on SVAD support.