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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.
- cardiovascular medicine
- heart failure
- cardiovascular system
- drugs: endocrine system
- congenital disorders
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Footnotes
Contributors CD acquired and analysed the data for the case report. He drafted and revised the report, including the final approval of the version to be published. He agrees to be accountable for all aspects of the work in relation to the accuracy or integrity of any part of the work. Mr JB made substantial contributions to the design and analysis of the work including revising it critically for important intellectual content and final approval of the version to be published. He also agrees to be accountable for all aspects of the work in relation to the accuracy or integrity of any part of the work. Mr JC made substantial contributions to the design and interpretation of the work including revising it critically for important intellectual content and final approval of the version to be published. He agrees to be accountable for all aspects of the work in relation to the accuracy or integrity of any part of the work. Dr KPW made substantial contributions to the design, analysis and interpretation of the work including revising it critically for important intellectual content and final approval of the version to be published. He agrees to be accountable for all aspects of the work in relation to the accuracy or integrity of any part of the work. The authors would also like to acknowledge the contribution of The Mater University Misericordiae University Hospital Histopathology Department and in particular, Dr Tom Nolis, who advised on the histopathology involved in the case.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.