Takotsubo syndrome and rheumatic diseases—a critical systematic review

Abstract

Since its description in 1990, Takotsubo syndrome (TTS), an acute cardiac condition triggered by physical or emotional stress, has been believed to be related to catecholamine surge from overwhelming sympathetic activity. While symptomatology, biochemical features, ECG and echocardiogram alterations are largely indistinguishable from acute coronary syndrome, the absence of culprit coronary lesions often necessitates further investigations, uncovering underlying inflammatory processes. Mechanistically, animal models of TTS reveal early neutrophil infiltration followed by staged ingression of two subtypes of macrophages (M1, M2) mediating initial acute inflammatory changes (M1), followed by switching to anti-inflammatory signals (M2) that enhance myocardial tissue recovery. Here, we begin with a description of two TTS patients with primary Sjögren’s syndrome and Takayasu’s arteritis, followed by a systematic literature review that summarizes the demographic and clinical features of TTS patients with rheumatological conditions. Potential impact of disease manifestations and treatment of rheumatological conditions on TTS are critically discussed.

Case Report: Acute Heart Failure Induced by the Combination of Takayasu's, Takotsubo and Coronary Vasospasm in an Elementary School Teacher–A Reaction to Return-to-Work Stress After COVID-19?

“…Other factors involved in the pathophysiology of TTS include estrogen deprivation, inflammation and immune activation ( 3 ). In line with the latter hypothesis, there is a potential link between rheumatological disorders and TTS ( 11 ). A retrospective epidemiologic study showed that the prevalence of TA among all TTS-related hospitalizations was 0.05%.…”

“…During admission, patients with TA and TTS had a higher rate of stroke, respiratory failure, necessity of mechanical ventilation and higher healthcare resource utilization, highlighting the possible role of TA in the prognosis of TTS ( 12 ). However, given the paucity of reports linking TA and other autoimmune diseases with TTS even though both diseases have a predilection for middle-aged women, it has been suggested that immunosuppressive treatment of these rheumatological disorders might protect against pro-inflammatory responses to catecholamine surge seen in TTS ( 11 ).…”

“…The sympathetic stimulation and subsequent catecholamine surge involved in TTS can cause acute myocardial dysfunction through a variety of mechanisms, including microvascular dysfunction, multi-vessel epicardial coronary artery spasm, direct myocyte toxicity and activation of cardiomyocyte preservation pathways by downregulating cellular metabolism ( 11 ).…”

Recurrent episodes of Takotsubo cardiomyopathy in systemic sclerosis

“…Autopsy studies have reported inflammatory, fibrotic as well as vascular myocardial abnormalities in 80% of SSc patients. 3 The diagnosis of primary cardiac disease, however, remains challenging as clinical findings of cardiac abnormalities in the early stage of disease are uncommon and, if present, very subtle and non-specific. 2 The diagnostic workup is further hampered due to the lack of criteria for distinguishing between primary cardiac involvement and other more common causes of cardiomyopathy such as hypertension and ischaemia.…”

“…4 However, recent investigation proposed an additional inflammatory pathway which might be relevant in the context of autoimmune rheumatic diseases. 3,4 Although TTS has traditionally been regarded as a benign, transient condition, it has recently been reported that acute mortality may be significant and patients can experience persistent symptoms, even though left ventricular function is completely normalized. 5 In addition, approximately 1% to 4% of patients will experience a recurrent episode of TTS, most commonly within 5 years after the first event.…”