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