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An Expanding Perspective: Extreme Aortic Dilation in a Patient With Single Ventricle Palliation
Kaitlyn Krebushevski1, Anders Jenson2, Aaron A Amundson3
1Department of Pediatric and Adolescent Medicine / Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.
World Journal for Pediatric & Congenital Heart Surgery
|September 25, 2025
Summary
Aortic dilation after single ventricle palliation can progress significantly, as seen in a Fontan circulation patient who required aortic root and valve replacement. Long-term surveillance is crucial for managing this complex condition.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Cardiovascular Surgery
Background:
- Aortic dilation is a known complication following single ventricle palliation.
- Long-term clinical implications of this dilation are not fully understood.
- Complex congenital heart disease requires specialized palliative and surgical approaches.
Purpose of the Study:
- To report a case of severe aortic dilation and stenosis in an adult with palliated single ventricle physiology.
- To highlight the need for vigilant monitoring and tailored management strategies.
- To emphasize the potential requirement for complex surgical intervention.
Main Methods:
- Case report of a 38-year-old male with complex congenital heart disease.
- Review of clinical history including Fontan circulation palliation.
- Description of diagnostic findings and surgical intervention (Bentall procedure).
Main Results:
- The patient developed severe neo-aortic root (81×65 mm) and arch dilation with aortic valve stenosis.
- Surgical intervention included a Bentall procedure, total arch replacement, and aortic valve replacement.
- This case illustrates the variable and potentially severe progression of aortic pathology.
Conclusions:
- Aortic dilation in single ventricle physiology is a progressive concern.
- Individualized surveillance and management are essential.
- Further research with larger cohorts is needed to guide surgical intervention criteria.
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