Takotsubo syndrome occurring in systemic diseases: A French multicenter retrospective case-control study and...

Describe the characteristics of patients presenting with TTS during the course of a broad spectrum of systemic diseases, in comparison to classic TTS.

French multicenter retrospective case-control study completed by a literature review.

19 new cases were included in the study. The literature review identified 25 previously published cases. Among the 44 patients, 41 were females, with a median age of 67 years. The main underlying systemic diseases were systemic lupus erythematosus for seven, rheumatoid arthritis for six and primary Sjögren's syndrome for five. A TTS trigger was found in 34 cases, including a systemic disease flare-up in 28. The flare-up was treated in 15 cases, mainly with corticosteroids. One patient died during the episode, unrelated to the TTS. With a median follow-up of 24 months, all patients had recovered a normal LVEF, one had presented a recurrence of TTS, and none had died of a cardiac cause. Finally, the 19 new patients were compared with 19 classic TTS. The disease characteristics were extremely similar, with no significant difference in terms of clinical, electrocardiographic, biological and echocardiographic presentation.

A broad spectrum of systemic diseases may rarely be accompanied by TTS, particularly during disease flare-ups. Although uncommon, TTS should be borne in mind in the presence of any cardiac symptomatology during the course of a systemic disease. Compared with classic TTS, their clinical, biological and echographic presentation is unremarkable. The prognosis for TTS appears to be good, with the consistent recovery of LVEF and no cardiac-related deaths.