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Quadricuspid aortic valve with centrally fenestrated leaflets causing aortic insufficiency.
W H Coltharp1, E T Warren, B J Heath
1Department of Surgery, Division of Cardiothoracic Surgery, University of Mississippi Medical Center, Jackson, Mississippi, USA.
Texas Heart Institute Journal
|January 1, 1988
Summary
A rare quadricuspid aortic valve with central leaflet fenestrations caused severe aortic insufficiency in a young man. This unique presentation, leading to aortic regurgitation, has not been previously reported in medical literature.
Area of Science:
- Cardiology
- Cardiac Surgery
- Medical Case Reports
Background:
- Quadricuspid aortic valve (QAV) is a rare congenital anomaly.
- Aortic insufficiency (AI) can result from various aortic valve pathologies.
- Fenestrations within aortic valve leaflets are an uncommon finding.
Purpose of the Study:
- To report a unique case of quadricuspid aortic valve with central leaflet fenestrations.
- To describe the clinical presentation and management of severe aortic regurgitation caused by this anomaly.
- To highlight a previously unreported cause of aortic insufficiency.
Main Methods:
- Case presentation of a 20-year-old male patient.
- Review of clinical history, echocardiographic findings, and surgical intervention.
- Description of the specific anatomical abnormalities of the aortic valve.
Main Results:
- The patient presented with severe aortic regurgitation (AR) and a history of AI since age 2.
- A quadricuspid aortic valve with a single, central leaflet fenestration was identified.
- Aortic valve replacement was performed due to severe AR, exacerbated by a flail leaflet.
Conclusions:
- This case represents the first reported instance of a quadricuspid aortic valve with central leaflet fenestrations causing significant aortic insufficiency.
- The combination of leaflet fenestrations and leaflet flail contributed to severe AR.
- This anomaly should be considered in the differential diagnosis of congenital aortic valve disease presenting with aortic regurgitation.