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Cardiovascular ochronosis.

J J Gaines, G M Pai

Archives of Pathology & Laboratory Medicine

|October 1, 1987

Summary

This summary is machine-generated.

Alkaptonuric ochronosis can cause aortic valve calcification and stenosis due to ochronotic pigment deposits. This ultrastructural study reveals pigment in degenerated cells, potentially stimulating dystrophic calcification.

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Area of Science:

Cardiovascular Pathology
Connective Tissue Diseases

Background:

Alkaptonuric ochronosis is a rare inherited disorder.
It is characterized by ochronosis, a bluish-black discoloration of connective tissues.
Aortic valve disease is a known complication.

Observation:

A patient with alkaptonuric ochronosis underwent aortic valve replacement and coronary artery bypass grafting.
Ochronosis of the aortic valve and aorta was observed during surgery.
Tissue samples were analyzed using light and transmission electron microscopy.

Findings:

Intracellular and extracellular ochronotic pigment deposits were found in the aortic valve.
Extracellular pigment was associated with degenerated cells and valvular calcification.
Ochronotic pigment was identified within macrophages and smooth muscle cells in the aorta.

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Implications:

Extracellular ochronotic pigment may trigger dystrophic calcification in the aortic valve.
This process could contribute to aortic valve calcification and stenosis in alkaptonuric ochronosis.
This is the first ultrastructural investigation of the aorta and aortic valve in this condition.