My Worst Nightmare: The Severely Regurgitant And Stenotic Truncal Valve
Morgan K. Moroi, MD, Edward Buratto, MD, PhD, Amee Shah, MD, Diana Vargas, MD, David M. Kalfa, MD, PhD.
Columbia University Irving Medical Center, New York Presbyterian Hospital, New York, NY, USA.
Objective(s): To describe the complex case of a child diagnosed with truncus arteriosus and severe truncal valve (TrV) dysfunction.
Methods: A 2.8kg 2-day-old male neonate was transferred to our institution in multiorgan failure following postnatal diagnosis of Type II truncus arteriosus with quadricuspid TrV with severe stenosis and severe regurgitation, proximal branch PA stenosis, Scimitar syndrome, bilateral SVCs, mesocardia, and severe RV dysfunction. On day-of-life 6, patient underwent high-risk truncus repair involving TrV tricuspidization and branch PA plasty.
Results: Post-repair TEE showed no residual lesion, trivial TrV regurgitation, trivial stenosis, and an RV pressure equal to one-third the systemic pressure. Multiorgan dysfunction began to improve. On POD14, TTE showed dehiscence of the TrV repair with severe regurgitation. Medical optimization and branch PA stenting on POD17 allowed for clinical stabilization, extubation, and growth. Patient was then "listed" for living allogenic double valve replacement. However, patient decompensated with worsening respiratory status. On POD177, patient underwent TrV re-repair, repeat bilateral PA plasty, and RV-PA conduit exchange, resulting in mild TrV regurgitation on postoperative TEE. After initial clinical improvement, patient exhibited signs of ischemia on POD198. Workup revealed intrinsic hypoplasia of the left coronary artery (LCA) as well as moderate TrV insufficiency. On POD203, patient underwent LCA patch augmentation and mechanical TrV replacement with annular root enlargement. Patient ultimately progressed to discharge to rehab on POD264 followed by home.
Conclusions: Management of neonates in multiorgan failure with truncus arteriosus, severe TrV regurgitation, and severe TrV stenosis remains challenging.
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