Abstract
Mast cell activation disease is a genetic disease entity characterized by a very pronounced clinical symptomatology with potential manifestations in virtually every organ and tissue. These arise from the inappropriate release of mast cell mediators and the accumulation of both morphologically normal and mutated mast cells. Owing to the high prevalence of the disease-estimated to be up to 17%-cardiologists and angiologists are frequently confronted with mast cell activation disease in their daily clinical practice, often without recognizing it. Therefore, every cardiologist and angiologist should possess basic knowledge about this disease and be aware of its cardiovascular challenges. This review summarizes the current state of knowledge on this highly complex disease, with a particular focus on the cardiovascular aspects.