Typical and reverse Takotsubo syndromes as initial manifestations of consecutive Addisonian crises in a 38-year-old patient: the heart has its reasons!

Abstract

We report an interesting case of a 38-year-old woman presenting with reverse Takotsubo syndrome (TTS) secondary to an Addisonian crisis, her second such episode. A few years prior, she had presented with typical TTS in the setting of Addisonian crisis; diagnostic work-up revealing Auto-Immune Polyglandular Syndrome Type II (APS II). We believe this to be the first case report of typical and variant phenotypes of TTS in a patient with APS II. The pathogenic link between these two conditions is explored. In patients presenting with Addisonian crises and refractory shock, the possibility of concurrent TTS should be considered. TTS muddies the diagnostic waters and poses therapeutic challenges as outlined.

Autoimmune polyendocrine syndrome II presenting paradoxically as Takotsubo cardiomyopathy: a case report and reappraisal of pathophysiology

“…However, one must remember that glucocorticoid therapy initiation is a doubleedged sword and it may have an unfavorable effect on myocardial contractility in some patients as hypothesized above. To the best of our knowledge, this is only the 5th case report in the literature in which TCM was associated with APS II [19][20][21][22].…”

COVID–19ワクチン接種後に副腎クリーゼ，たこつぼ心筋症を発症しショックとなり多腺性自己免疫症候群2型の診断に至った1例(A patient with post COVID–19 vaccination presented with convulsions and cardiogenic shock due to adrenal crisis and Takotsubo cardiomyopathy diagnosed with autoimmune polyendocrine syndromes type II and Addison’s disease)

“…本症例のように，副腎不全やAPS 2型にたこつぼ心筋症を合併する例は少数ながら複数の報告がある 9) , 10) , 11) 。機序として①糖質コルチコイドによる心筋保護のない環境下でのカテコラミン暴露が有害となる，②糖質コルチコイドが心筋細胞膜のカルシウム輸送機能の維持に必要である，③心筋小胞体でのphosphorylase活性低下により，グリコーゲン分解が傷害されると心筋興奮収縮の異常を来すとされる 11) 。また，たこつぼ心筋症は性ホルモン異常が誘因になりうることが知られていて，閉経後女性に多い。本症例も早発閉経を認めており，発症の素因となった可能性がある。既報告例のいずれもステロイド補充にてたこつぼ心筋症の軽快を認めており，副腎クリーゼの早期認知およびステロイド補充が肝要と考えられる。…”