AN UNUSUAL CAUSE OF MITRAL VALVE THICKENING: COGAN SYNDROME

INRODUCTION

Cogan syndrome is an autoimmune disorder characterized with ocular and vestibular symptoms and only minority (less than 15%) of patients suffer from systemic disease which is explained by autoimmuune vasculitis.(1) Cardiovascular involvement of Cogan syndrome is rare and effects mostly subclavian and carotid arteries, aortic valve or aorta. Cogan syndrome is similar to Takayasu arteritis in terms of cardiovascular involvement. In current literature, proximal aortic vasculitis, aortic valve perforation, left main coronary artery stenosis or even renal artery stenosis were reported(2,3); however, mitral valve involvement is rare.In current literature, only one case report mentioned how Cogan syndrome may effect mitral valve. (4) Below, we report a patient with Cogan syndrome presenting moderate to severe mitral regurgitation with severe thickening of both mitral leaflets. CASE REPORT

39 year old male patient had admitted to our outpatient clinic complaining of shortness of breath more than a year. He had previous history of Cogan syndrome, receiving immunosupressant treatments and aortic valve implantation in 2012. Mechanical aortic valve implantation was done because of severe aortic insufficency with unknown aetioloy. Immediate after this operation, the patient suffered from infective endocarditis,afterwards periprosthetic abcess formation occured; thus re-operation was decided.Benthall operation was performed in the same month.His physical examination was normal except for presence of red eye sign. His current transthorasic echocardiography demonstrated massively thickened mitral leaflets as well as moderate to severe mitral regurgitation. Coronary angiography did not demonstrate any critical stenosis in coronary arteries. Transoesaphageal echocardiography revealed moderate to severe mitral regurgitation, severe thickening in both mitral leaflets and and mild restriction in leaflet motions.(figure 1, figure 2) This patient is dicussed by Heart Team and surgery was decided if the patient had dyspnea or any other symptoms under appropriate medical treatment.

DISCUSSION Cogan syndrome is a vasculitis which effects medium sized arteries, patients may represent subclavian or carotid arteritis, aortitis, myocardial infarction or even renal artery stenosis.The main pathophysiological causes of mitral valve thickening in Cogan syndrome are mononuclear cell infiltration,multiple microabscesses, and myxoid degeneration.(4) From the data we gained so far, we suppose that this patient may have undergone aortic valve implantation because of Cogan’s cardiovascular involvement in 2012.

Mitral valve thickening can develop in rheumatic valve disease course or after receiving certain medical treatments,including bromcriptine.(5) Out patient did not receve any dopamine agonists ,including cabergoline and bromocriptine ,during his lifetime. In addition mitral valve thickening may also develop in the course of rheumatic mitral valve disease.In this patient, apart from rheumatic valve disease, both leaflets were affected, no calcification in valvular apparatus or lack of chordal shortening were observed. Afterall, it was decided by our Heart Team that the main pathology causing mitral valve thickening was Cogan syndrome.

To the best our knowledge, our patient is a rare example of mitral valve involvement in patients with Cogan syndrome.

Data Availability Statement The data that demonstrate the findings of this case are available from the corresponding author upon reasonable request.

Ethical approval statement and informed consent are not applicable for my case report.

REFERENCES

1-Cogan’s Syndrome: Clinical Presentations and Update on Treatment, Gabriela Mabel Espinoza1 & Joseph Wheeler2 & Katherine K. Temprano3 & Angela Prost Keller1, Current Allergy and Asthma Reports, doi: 10.1007/s11882-020-00945-1.

2-Late-onset Cogan’s syndrome associated with large-vessel vasculitis, Rodriguez et al, Reumatol Clin (Engl Ed), Sep-Oct 2019;15(5):e30-e32. doi: 10.1016/j.reuma.2017.05.002