Therefore, our research was oriented toward searching separately for the two intricate pathologies, the review encompassing medical articles describing, on one hand, blood culture-negative infective endocarditis with Coxiella burnetii on a mechanical aortic valve and, on the other hand, studies presenting patients having familial thoracic aortic aneurysm and/or dissection due to the ACTA2 disease-causing variant. Here, ACTA2 is linked to infective endocarditis.