Tako-TSUBO Cardiomyopathy

Inflammatory Cardiovascular Diseases and Autoimmune Rheumatic Diseases (ICARDs) encompass cardiovascular involvement in connective tissue diseases, vasculitis, and primary inflammatory cardiac processes affecting all layers of the heart. ICARDs are associated with increased cardiovascular morbidity and mortality, independently of traditional risk factors, via multiple pathophysiological mechanisms. Diagnosis and prognosis are challenged by the heterogeneity of clinical presentations. Multimodality cardiovascular imaging - including cardiovascular magnetic resonance (CMR), transthoracic echocardiography, and positron emission tomography (PET) - plays a central role in detecting and characterizing inflammatory involvement, and may offer prognostic insights. Given the limited data on the diagnostic and prognostic utility of these imaging modalities in ICARDs, the EACVI-INFLAME study aims to assess the prevalence of confirmed cardiovascular involvement in patients with suspected or established ICARDs undergoing CMR and/or cardiac PET in a multicentric international cohort.

The underlying mechanisms of microvascular dysfunction in Takotsubo cardiomyopathy remain incompletely understood. As CD34+ cells are essential to coronary microvascular homeostasis we will investigate the potential association between CD34+ cell count and changes in left ventricular function in patients with Takotsubo cardiomyopathy at baseline and 6-month follow-up.

Takotsubo syndrome is a condition which mimics acute myocardial infarction, and is diagnosed in 1.5% to 2.2% of patients referred to hospital with suspected acute coronary syndrome. It is also known as broken heart syndrome, takotsubo cardiomyopathy, stress cardiomyopathy and apical ballooning cardiomyopathy, among other names. The pathogenesis of this disorder is not well understood. Possible mechanisms include catecholamine excess, coronary artery spasm, microvascular dysfunction, among others. This is a multicenter, nation-wide, observational study of patients who were previously diagnosed with takotsubo syndrome. The investigators aim to use this registry to help plan and carry out further studies and to improve understanding of the pathophysiologic mechanisms of this syndrome. In addition participants will be followed for events, and to monitor quality of life and stress.

Heart scarring, also known as fibrosis, plays a major role in a lot of heart muscle abnormalities. These abnormalities of the heart muscle can lead to major issues such as symptoms of heart failure, dangerous heart rhythm disturbances and even death. However, a lot of these conditions are still not fully understood and treatment options are limited. We here aim to use a new radioactive dye called 68Ga-FAPI to identify patterns and the activity of heart muscle scarring. This radioactive dye is being used in humans particularly in identifying and monitoring cancers and has shown promise in identifying scarring in the heart as well. This will help us not only understand the underlying disease process and risk stratify these patients but also potentially help us develop new targeted therapies that can affect heart muscle scarring. Participants will undergo a baseline MRI scan using this new dye and a plain MRI scan will repeated 12-18 months after to see if there are any changes in the process.

Consecutive consenting patients over the age of 18 years diagnosed with Takotsubo syndrome according to the position statement of the European Journal of Heart Failure (2016) will be included in the study. The primary endpoint is to evaluate the incidence of coronary microcirculation dysfunction and its correlation with the clinical presentation and the prognosis in patients with TAKO-Tsubo syndrome. Secondary endpoints will be to assess the incidence of in-hospital mortality, the prevalence of cardiogenic shock, the correlation between the levels of biomarkers analyzed, microvascular dysfunction, perceived quality of life, and angina severity during hospitalization and at subsequent follow-ups, and the influence of knowledge of the level of microvascular dysfunction on clinical management and prescribed therapies. Enrolment: 2 years, follow-up: 1 month and 1 year. Total: 3 years.