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Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Profound systemic hypoperfusion in a patient with ventricular inversion temporised with transcatheter ductal closure
Sally Hunt1, James Ryan Shea2, Kieran G Leong1
1Department of Pediatrics, https://ror.org/00qz24g20The University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, NC, US.
Abstract:
Isolated atrioventricular discordance with ventriculoarterial concordance, or ventricular inversion, is a rare congenital cardiac anomaly that produces transposition-like physiology. We report a case of prenatally diagnosed ventricular inversion presenting with profound systemic hypoperfusion secondary to a large patent ductus arteriosus (PDA). Initial management included prostaglandin E1 to maintain ductal patency and balloon atrial septostomy to promote intracardiac mixing. Despite these measures, the patient developed significant pulmonary overcirculation and systemic steal, necessitating urgent transcatheter PDA closure. This intervention resulted in immediate hemodynamic improvement and stabilization, allowing for subsequent definitive repair with VSD closure and Senning atrial switch. The case underscores the importance of individualized hemodynamic assessment and the potential for transcatheter ductal closure to temporize systemic perfusion in select patients with ventricular inversion prior to anatomic correction.
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