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Hypertensive Emergency Secondary to Paraganglioma in a Pediatric Patient With a Fenestrated Fontan
Kara Gay-Simon1, Kathryn Forbes1, Jose Ugarriza Mendoza1
1Department of Cardiology, Cardiac Critical Care Medicine at Nicklaus Children's Hospital, Miami, Florida, USA.
Insights
Children with Fontan physiology face risks of secondary hypertension. A rare paraganglioma, a neuroendocrine tumor, was identified as a cause in a young patient, highlighting the need for vigilance.
Area of Science:
- Pediatric Cardiology
- Pediatric Endocrinology
- Surgical Oncology
Background:
- Children with Fontan physiology are susceptible to serious complications, including secondary hypertension.
- Congenital heart disease (CHD) can predispose patients to various health issues.
Observation:
- A 10-year-old girl with hypoplastic left heart syndrome post-Fontan presented with hypertensive crisis, chest pain, and atrial tachycardia.
- Diagnostic work-up revealed elevated cardiac biomarkers, a retroperitoneal mass, and significantly elevated catecholamines, confirming a catecholamine-secreting paraganglioma.
Findings:
- Paragangliomas, though rare in children, may be associated with chronic hypoxia in CHD.
- Sustained hypoxemia in Fontan patients might increase the risk of developing neuroendocrine tumors.
Implications:
- This case underscores the importance of vigilance for secondary causes of hypertension in Fontan patients.
- Early recognition and multidisciplinary care are crucial for managing secondary hypertension in pediatric CHD.
Background:
Children with Fontan physiology are at risk for a range of complications, including potentially life-threatening causes of secondary hypertension.
Case Summary:
We present a 10-year-old girl with hypoplastic left heart syndrome post-Fontan who developed hypertensive crisis, chest pain, and atrial tachycardia. Work-up revealed elevated cardiac biomarkers and a retroperitoneal mass at the aortic bifurcation. Laboratory testing showed markedly elevated catecholamines, and imaging confirmed a catecholamine-secreting paraganglioma. She was medically stabilized with alpha and beta blockade, and then underwent successful surgical resection. She was discharged with well-controlled blood pressure and continues to receive multidisciplinary follow-up.
Discussion:
Paragangliomas are rare in children but may be linked to chronic hypoxia in congenital heart disease. In Fontan patients, sustained hypoxemia may predispose to neuroendocrine tumors, necessitating vigilance for secondary causes of hypertension.
Take-Home Message:
Early recognition and coordinated care are essential in managing secondary hypertension in children with complex congenital heart disease.
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