Truncal Valve Repair in Neonates Using Pericardial Leaflet Extension Simin Bahrami, MD,* Fotios Mitropoulos, MD,* Frederick Leong, MD, † Daniel S. Levi, MD, † Hillel Laks, MD,* and Mark D. Plunkett, MD* ‡ *Division of Cardiothoracic Surgery, Department of Surgery, and † Division of Pediatric Cardiology, Department of Pediatrics, Ronald Reagan UCLA Medical Center, University of California, Los Angeles, Calif, and ‡ University of Kentucky College of Medicine, Lexington, Ky, USA ABSTRACT Truncal valve insufficiency is a significant risk factor for post-operative mortality following repair of truncus arteriosus. The surgical management of dysplastic and insufficient truncal valves remains an operative challenge. We report the cases of two infants with type 2 truncus arteriosus and severely dysplastic and insufficient quadricuspid truncal valves. At primary repair, their truncal valves were successfully repaired using pericardial leaflet extensions. This technique may be used in neonates with truncal valve insufficiency as part of the primary repair of truncus arteriosus. Key Words. Congenital Heart Disease; Truncus Arteriosus; Aortic Valve; Neonate Introduction D ue to advances in surgical techniques, neo- nates with truncus arteriosus can undergo primary complete repair with low morbidity and mortality. However, infants with truncal valve insufficiency are at significantly increased risk for operative and late mortality. The repair of the truncal valve is often individualized based on the morphology of the valve in each patient. In neo- nates with extremely dysplastic and insufficient truncal valves, replacement with a homograft may be necessary. We report two patients who under- went truncus arteriosus repair with the repair of insufficient truncal valves using the technique of pericardial leaflet extensions. Case Report Two neonates presented with murmurs during their first day of life. Echocardiography revealed type 2 truncus arteriosus defects in both patients. Each was found to have a dysplastic quadricu- spid truncal valve with mild stenosis and severe insufficiency. The first patient was taken to the operating room on the ninth day of life for complete repair. The truncal valve was found to be quadricuspid with dysplastic fibrous tissue at the edge of each leaflet. The leading edge of each leaflet was excised back to normal tissue. Two of the leaflets were sutured together to form a single leaflet. Native pericardium was treated with 0.6% glut- araldehyde for 5 minutes and used to make leaflet extensions (Figure 1). The resulting three-leaflet valve with pericardial extensions was tested with saline and found to be competent with improved coaptation and no evidence of stenosis. The repair was then completed with closure of the ventricular and atrial septal defects and placement of an aortic homograft from the right ventricle to the pul- monary arteries. Intraoperative transesophageal echocardiography confirmed competency of the repaired valve with improved coaptation and no evidence of stenosis. His post-operative course was uncomplicated, and he was discharged home on post-operative day 12. He did clinically well until about one year of age when he was readmitted for fungemia requiring 8 weeks of parenteral anti-fungal therapy. Following treatment, an echocardiogram revealed recurrent mild to moderate aortic steno- sis and severe aortic insufficiency. He was taken to the operating room where the repaired valve was found to be fibrotic with fused commissures. No 281 © 2009 Copyright the Authors Journal Compilation © 2009 Wiley Periodicals, Inc. Congenit Heart Dis. 2009;4:281–283