| Summary: | Quadricuspid aortic valve is a very rare congenital anomaly. Its association with rheumatoid arthritis is exceptional with this being the third case reported in the literature. We report a case of a 52 year old female patient with quadricuspid aortic valve type C accompanied by moderate to severe aortic regurgitation and longstanding, advanced form of rheumatoid arthritis. Having refused surgical aortic valve intervention 4 years ago, the patient is currently under a watchful follow-up strategy. The patient received a diagnosis of rheumatoid arthritis over 15 years before and presently has serious deformities in the hands, legs, feet, and spine. In conclusion, quadricuspid aortic valve and rheumatoid arthritis together are extremely rare. While it is possible that this association is coincidental, considering the genetic background of both disorders, there is a potential for them to be interconnected comorbidities. This report is the first to highlight the association between the 2 disorders.
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