Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus

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ORIGINAL ARTICLE

Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus Adnan M. Bakar1   · John M. Costello2,5 · Peter Sassalos3 · Venu Amula4 · Jason R. Buckley5 · Arthur J. Smerling6 · Ilias Iliopoulos7 · Christine M. Riley8 · Aimee Jennings9 · Katherine Cashen10 · Sukumar Suguna Narasimhulu11 · Keshava Murthy Narayana Gowda12 · Michael Wilhelm13 · Aditya Badheka14 · James E. Slaven15 · Christopher W. Mastropietro16 · the Collaborative Research from the Pediatric Cardiac Intensive Care Society (CoRePCICS) Investigators Received: 28 February 2020 / Accepted: 25 June 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020

Abstract Truncal valve management in patients with truncus arteriosus is a clinical challenge, and indications for truncal valve intervention have not been defined. We sought to evaluate truncal valve dysfunction and primary valve intervention in patients with truncus arteriosus and determine risk factors for later truncal valve intervention. We conducted a retrospective cohort study of children who underwent truncus arteriosus repair at 15 centers between 2009 and 2016. Multivariable competing risk analysis was performed to determine risk factors for later truncal valve intervention. We reviewed 252 patients. Forty-two patients (17%) underwent truncal valve intervention during their initial surgery. Postoperative extracorporeal support, CPR, and operative mortality for patients who underwent truncal valve interventions were statistically similar to the rest of the cohort. Truncal valve interventions were performed in 5 of 64 patients with mild insufficiency; 5 of 16 patients with mild-tomoderate insufficiency; 17 of 35 patients with moderate insufficiency; 5 of 9 patients with moderate-to-severe insufficiency; and all 10 patients with severe insufficiency. Twenty patients (8%) underwent later truncal valve intervention, five of whom had no truncal valve intervention during initial surgical repair. Multivariable analysis revealed truncal valve intervention during initial repair (HR 11.5; 95% CI 2.5, 53.2) and moderate or greater truncal insufficiency prior to initial repair (HR 4.0; 95% CI 1.1, 14.5) to be independently associated with later truncal valve intervention. In conclusion, in a multicenter cohort of children with truncus arteriosus, 17% had truncal valve intervention during initial surgical repair. For patients in whom variable truncal valve insufficiency is present and primary intervention was not performed, late interventions were uncommon. Conservative surgical approach to truncal valve management may be justifiable. Keywords  Congenital heart defects · Truncus arteriosus · Cardiac valves · Risk factors

Introduction Truncus arteriosus is a heterogeneous form of congenital heart defect with many possible anatomic abnormalities of the truncal valve and other cardiac structures that can complicate surgical management. Frequently found associations include an abnormal number of truncal valve cusps, differing Electronic supp