https://orcid.org/0000-0003-4217-9400
A 69-year-old woman with hypertension, type 2 diabetes, and anaemia presented with progressive exertional breathlessness. Clinical examination revealed a rumbling diastolic cardiac murmur best audible at the apex. Transthoracic and transoesophageal echocardiography showed heavy calcification of the mitral valve (MV) annulus, extending down both MV leaflets, and causing severe MV stenosis (MS) (valve area 0.6–0.8 cm2) (Panel A; Supplementary data online, Videos S1 and S2). Biventricular systolic function was normal; there was no significant mitral regurgitation or pulmonary hypertension. A computed tomography coronary angiogram was performed to exclude coronary artery disease. Coronary arteries were unobstructed. There was severe mitral annular calcification (MAC) extending into the left ventricular myocardium (Panels B–D) in a very unusual pattern of finger-like structures on 3D volume-rendered images (Panels E–G). There was also suspicion of calcification of some of the coronary venous structures. An invasive coronary angiogram showed the extensive myocardial calcification (Panels H and I; Supplementary data online, Videos S3–S5) and excluded any coronary vein calcification. Conventional surgical MV replacement was judged unsuitable due to unfavourable MV anatomy; the patient is being considered for transcatheter MV replacement when the technology becomes more established. While MAC is a common finding in cardiovascular imaging studies, with an increasing prevalence with advanced age, female sex, and comorbidities, intramyocardial calcification associated with MAC or not is rare phenomenon. This case illustrates a rare pattern of extensive intramyocardial calcification and severe MS secondary to severe MAC.
Supplementary data are available at European Heart Journal - Cardiovascular Imaging online.
