Abstract

We report a case of a 16-year-old adolescent male born with univentricular congenital cyanotic heart disease (CCHD) who was diagnosed with an incidental paraganglioma while awaiting a cardiac transplant. The coexistence of paraganglioma and univentricular CCHD is very rare, with no previous cases described in the literature of a patient concurrently requiring a cardiac transplant. The complex physiology associated with a common atrium, common ventricle, aortopulmonary lung perfusion and a hypoplastic left lung rendered our patient extremely vulnerable to catecholamine-mediated effects of preload, contractility and afterload. The interactions and interdependence between these systems provided unique difficulties for perioperative management with serious implications for prospective cardiac transplant.