Idiopathic Myocardial Helical Calcification

A 71-year-old woman with a history of hypertension and hyperlipidemia was referred to Fukuoka Wajiro Hospital for investigation of echocardiographic abnormalities. Six months earlier, she had presented to another hospital with transient palpitations and been prescribed a phosphodiesterase (PDE) 5 inhibitor with no further examination.

Echocardiography showed a diffuse high echo area in the left ventricle (LV) and possible myocardial calcification. LV diastolic function was depressed, whereas systolic function was normal, with an ejection fraction of 65%. Despite the calcification, the patient had no valvular stenosis or regurgitation. A chest radiograph showed clear lung fields and diffuse calcification within the cardiac silhouette (Figure A). Diffuse calcific infiltration of the LV myocardium, which also involved the papillary muscles, mitral chordal apparatus, and mitral annulus, was revealed by 320-detector row computed tomography (CT). There was no calcification in the right heart. The LV calcification was helical and moved like a coiled spring (Figure B–D; Supplementary Movie). Coronary CT and angiography showed stenosis of the left anterior descending artery and total occlusion of the right coronary artery. Serum parathyroid hormone, calcium, and creatinine concentrations were within normal limits.

Figure.

(A) Chest radiograph and (B) 320-detector row computed tomography showing diffuse calcification within the cardiac silhouette. (C,D) The calcification of the left ventricle was helical and moved like a coiled spring.

Our differential diagnosis was myocardial infarction, cardiomyopathy, malignant tumor, or calcified amorphous tumor.¹

This a report of the rare entity of massive LV calcification visualized on cardiac CT in a patient with no relevant history.

The calcification did not match the ischemic area, so the etiology of the calcification was unclear. However, the helical structure matches the myocardial band for contraction,² and it is likely that the structure was possibly a dystrophic or amorphous tumor. Myocardial calcification may damage LV structure, resulting in restrictive cardiomyopathy that requires management and can progress, so should be followed-up carefully.

Disclosures

The authors have no financial conflicts of interest to disclose concerning the study.

Supplementary Files

Supplementary Movie. The LV calcification was helical structure and moved like a coiled spring.

Please find supplementary file(s);

http://dx.doi.org/10.1253/circrep.CR-20-0059

References

• 1.   Ho HH, Min JK, Lin F, Wong SC, Bergman G. Calcified amorphous tumor of the heart. Circulation 2008; 117: e171–e172.
• 2.   MacIver DH, Stephenson RS, Jensen B, Agger P, Sánchez-Quintana D, Jarvis JC, et al. The end of the unique myocardial band: Part 1. Anatomical considerations. Eur J Cardiothorac Surg 2018; 53: 112–119.