Kodakkandla Rakesh Kumar, Sarraju Venkata Sreedatta, Ayalasomayajula Nagesh
Department of Cardiovascular and Thoracic Surgery, Care Hospitals, Nampally, Hyderabad, India.
SAGE Open Med Case Rep. 2025 Jan 30;13:2050313X251316992. doi: 10.1177/2050313X251316992. eCollection 2025.
Myxomatous degeneration of a valve is a non-inflammatory degenerative process due to the disruption of the fibrous layer of the valve with mucopolysaccharide accumulation. It is mostly seen in the mitral valve but involvement in only aortic valve is rare. Here we present a case report of a 37-year-old man who came with shortness of breath for the past three months and was diagnosed with severe aortic regurgitation due to myxomatous aortic valve causing rupture of the non-coronary and right coronary cusp. After preoperative workup, he was taken for aortic valve replacement surgery. Intraoperative findings confirmed the rupture of right and non-coronary cusps from their annular attachment and the myxomatous nature of the aortic valve. Histopathological examination confirmed the myxomatous changes in the valve. The patient underwent aortic valve replacement with a 23 mm TTK Chitra valve and on follow-up, he is doing well. Although aortic valve regurgitation is a common disease, myxomatous degeneration of isolated aortic valve leading to aortic regurgitation is rare. This patient did not have any signs of connective tissue disorders like Marfan's syndrome, Ehlers-Danlos, etc., but presented with myxomatous aortic valve throwing an insight into further research into this rare disease.
瓣膜的黏液样变性是一种非炎症性退行性过程，由于瓣膜纤维层的破坏伴有黏多糖积聚。它最常见于二尖瓣，但仅累及主动脉瓣的情况罕见。在此，我们报告一例37岁男性病例，该患者在过去三个月出现气短症状，被诊断为严重主动脉瓣反流，病因是黏液样主动脉瓣导致无冠瓣和右冠瓣破裂。经过术前检查后，他接受了主动脉瓣置换手术。术中发现证实右冠瓣和无冠瓣从其瓣环附着处破裂以及主动脉瓣的黏液样性质。组织病理学检查证实了瓣膜的黏液样改变。该患者接受了23毫米TTK Chitra瓣膜的主动脉瓣置换术，随访时情况良好。虽然主动脉瓣反流是一种常见疾病，但孤立性主动脉瓣黏液样变性导致主动脉瓣反流却很罕见。该患者没有任何马凡综合征、埃勒斯-当洛综合征等结缔组织疾病的迹象，但出现了黏液样主动脉瓣，这为进一步研究这种罕见疾病提供了线索。
