ABSTRACT
Massive myocardial calcification is a very rare finding. Accurate
identification and characterization may help the clinicians to determine
the etiology and clinical significance. In this case, the diagnostic
pathway excluded previous myocardial infarction, myocarditis and
calcium-phosphate disorders. A possible dystrophic etiology was
considered. There are no standardized imaging features available to
classify specific subtypes of intramyocardial calcifications. The
relative merit of cardiac computed tomography and magnetic resonance
imaging in providing complimentary diagnostic information for calcific
myocardial lesions is shown. Knowledge of the potential etiology and
their imaging patterns are important to provide a concise and accurate
differential diagnosis.
An 81-year-old woman, affected by Horton disease and with previous
history of rheumatic fever, was hospitalized for acute chest pain.
Physical examination and EKG were not significant. Chest X-Ray showed a
diffuse hypodense lobulated area in the left ventricle (Figure 1: A).
The echocardiogram revealed diffuse aortic and mitral calcifications
with mild stenosis, marked septum and antero-lateral asymmetric
hypertrophy, with extensive antero-lateral calcifications (Figure 1: B,
Video 1). Non-contrast computed-tomography (CT) highlighted widespread
amorphous confluent calcifications in the left ventricular wall, with
septal sparing, extended to the mitral-aortic annulus and both coronary
arteries (Figure 1: C-C1 showing VRT reconstruction and C2 MPR
axial-view, Video 2). Cardiac magnetic resonance 3,0 T presented the
following: septal hypertrophy, antero-lateral wall thickening with areas
of intramyocardial signal-alteration, surrounded by normal myocardium
matching the CT calcifications. Areas of low-signal were shown in
multiple sequences: SSFP-cine in 4-chambers [4C] (Figure 2: D, Video
3) and in short-axis [SAX] (Figure 2: D1); T1-weighted-spin-echo in
4C (Figure 2: E) and SAX (Figure 2: E1); STIR in 4C (Figure 2: F).
T1-native-mapping in 4C focused on diffuse septum and lateral fibrosis
with low-signal (550 ms, normal range: 1150-1250 ms) compatible with
calcifications (Figure 2: G). After contrast-gadolinium injection,
PSIR-sequence showed intramyocardial hypersignal in the lateral wall
both in 4C and SAX, Figure 2: H-H1 respectively). Coronary angiography
documented significant disease that required revascularization.
Extensive intramyocardial calcifications are extremely rare (1). In our
case the diagnostic pathway excluded previous myocardial infarction,
myocarditis and calcium-phosphate disorders. A possible dystrophic
etiology was considered according also to the rheumatic disease. There
are no standardized imaging features available to classify specific
subtypes of intramyocardial calcifications (2). The combination of
imaging pattern and potential etiology plays a key role in
characterizing calcifications and deriving its clinical
impact.