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Paraganglioma and phaeochromocytoma in adult Fontan patients
Stephanie Bm Tan1, Maria Luz Garagiola2, Xiaoyang Liu1
1Department of Medical Imaging, University Health Network, JDMI, Toronto, Ontario, Canada.
Insights
Fontan procedure patients have an increased risk of developing phaeochromocytoma and paraganglioma (PPGL). This study found a 0.5% incidence of PPGL in adult Fontan patients, highlighting the need for vigilance.
Area of Science:
- Cardiology
- Oncology
- Radiology
Background:
- Fontan procedure is a palliative surgery for single ventricle congenital heart disease.
- Fontan patients face increased risks of extracardiac complications, including PPGL.
- Literature suggests a higher incidence of PPGL in Fontan circulation patients.
Purpose of the Study:
- To investigate the incidence, presentation, and imaging characteristics of PPGL in adult Fontan patients.
- To address the knowledge gap regarding PPGL in this specific patient population.
- To evaluate the largest North American cohort of adult Fontan patients.
Main Methods:
- Retrospective single-center study of adult Fontan patients (September 2015 - September 2022).
- Inclusion of all adult patients treated at a tertiary referral center for adult congenital heart disease.
- Retrospective search of imaging and electronic medical records for PPGL presence.
Main Results:
- Three out of 547 Fontan patients (0.5% incidence) were diagnosed with PPGL.
- Two patients had paraganglioma, and one had phaeochromocytoma; all were female.
- Median age at PPGL diagnosis was 19 years, with a median of 12 years post-Fontan operation.
Conclusions:
- Fontan patients exhibit an elevated risk for PPGL development.
- Clinical diagnosis of PPGL in Fontan patients is challenging due to confounding symptoms and markers.
- Radiological imaging is crucial for typical PPGL identification in this population.
Introduction:
The purpose of this study was to address the gap in knowledge in the incidence, presentation and imaging features of PPGL in Fontan patients. It has been hypothesised in the literature that Fontan circulation patients have an increased incidence of these tumours. This study is the largest cohort of adult Fontan patients in North America. The Fontan procedure is a palliative procedure for single ventricle congenital heart disease where the systemic venous return is directed into the pulmonary circulation. Fontan patients are at increased risk of developing several extracardiac complications including phaeochromocytoma and paraganglioma (PPGL).
Methods:
This retrospective single-centre study includes all adult patients who were treated between 1 September 2015 and 1 September 2022 in a tertiary referral centre for adult congenital heart disease. Imaging and electronic medical records were retrospectively searched for the presence of PPGL.
Results:
Five hundred and forty-seven Fontan patients were included in the study. Three patients had PPGL with an incidence of 0.5%. Two patients were diagnosed with paraganglioma and one with phaeochromocytoma. All patients were female. Median age of Fontan procedure was 1.8 years (range: 1.75-27 years). Median age of PPGL diagnosis was 19 years (range: 12-39 years). Median time from Fontan operation to diagnosis of PPGL was 12 years (range: 10.3-17.2 years).
Conclusion:
Fontan patients are at increased risk of PPGL. Clinically, the diagnosis of PPGL is challenging as symptoms and serum markers can be confounding in this population; however, the imaging features of PPGL remain typical making the role of the radiologist crucial.
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