Key result
Open transperitoneal excision of an incidental paraganglioma was successfully performed in a 16-year-old male with univentricular congenital cyanotic heart disease, enabling a subsequent successful orthotopic heart transplant.
Why the study?
The coexistence of paraganglioma and univentricular congenital cyanotic heart disease is very rare, with no previous cases described in a patient awaiting cardiac transplantation.
Case Report (n=1)
No
This case demonstrates the successful perioperative management and excision of an incidental paraganglioma in a patient with univentricular CCHD, enabling a subsequent successful heart transplant.
Demonstrates feasibility of paraganglioma excision enabling transplant in univentricular heart disease; hypothesis-generating from Level 3 evidence and should not yet change practice.
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.
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Doyle et al. (2021) conducted a case report in Paraganglioma and univentricular congenital cyanotic heart disease (n=1). Open transperitoneal excision of paraganglioma was evaluated on Clinical outcome (tumor excision and cardiac transplant). Open transperitoneal excision of an incidental paraganglioma was successfully performed in a 16-year-old male with univentricular congenital cyanotic heart disease, enabling a subsequent successful orthotopic heart transplant.
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