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Coronary arteriomegaly in a patient with Ehlers-Danlos syndrome and multiple aneurysms--a case report
Robert S Dieter1, Thomas Murtaugh, Jennifer Black
1William S. Middleton Veterans Hospital, Madison, and University of Wisconsin-Madison, 53705, USA.
Insights
This study presents a rare case of coronary arteriomegaly in a patient with Ehlers-Danlos syndrome and multiple aneurysms. The findings suggest a potential new vascular syndrome, distinct from known Ehlers-Danlos types.
Area of Science:
- Cardiology
- Genetics
- Vascular Biology
Background:
- Ehlers-Danlos syndrome is a group of inherited connective tissue disorders.
- Vascular complications, including aneurysms and dissections, are known manifestations.
- Coronary artery abnormalities are less commonly reported in Ehlers-Danlos syndrome.
Observation:
- A patient with Ehlers-Danlos syndrome and multiple aneurysms presented with myocardial infarction.
- Coronary arteriography showed distal vessel pruning without signs of occlusion.
- Fibroblast cultures ruled out Ehlers-Danlos syndrome types IV and VII.
Findings:
- The case exhibits coronary arteriomegaly, a significant dilation of coronary arteries.
- The distal "pruning" pattern is unusual and not typical of obstructive coronary artery disease.
- Genetic testing excluded specific, commonly associated Ehlers-Danlos syndrome subtypes.
Implications:
- This case may represent a previously unrecognized vascular syndrome.
- Further research is needed to characterize this potential new syndrome.
- Understanding this condition could improve diagnosis and management of vascular complications in related genetic disorders.
Abstract:
The authors report a case of coronary arteriomegaly in a patient with Ehlers-Danlos syndrome and multiple aneurysms who presented with myocardial infarction. Coronary arteriography revealed distal "pruning" of vessels without occlusive disease. Fibroblast cultures excluded Ehlers-Danlos syndrome types IV and VII. Literature review suggests this may represent a previously unidentified vascular syndrome.
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