This case was a 70-year-old patient who had a history of atrial fibrillation (AF), symptomatic of dyspnea and congestive heart failure. At that time, his AF was converted by electrical cardioversion with resolution of his symptoms. A year later, he presented with several months' history of progressive and marked dyspnea with minimal exertion. He underwent regadenoson MPI for cardiac evaluation. His electrocardiogram (ECG) showed a normal sinus rhythm, first degree atrioventricular (AV) block, and T wave inversion in the inferolateral leads suggestive of myocardial ischemia (Figure 1). His MPI demonstrated mildly decreased LV ejection fraction of 49% without evidence of myocardial ischemia or infarction; however, significant LV calcification (Figure 2) was observed on the low-dose CT used for attenuation correction during MPI.
Subsequently, other imaging studies, including chest x-ray, echocardiography, and chest CT, were obtained for further evaluation. The chest x-ray (Figure 3) showed extensive cardiac calcification, and the echocardiogram (Figure 4) revealed significant calcification of mitral valve annulus, LV septum, and mid free wall. However, his chest CT (Figure 5) revealed massive myocardial calcification involving only LV and its overlaying pericardium.
The patient had no past history of malignancy, endocrine disorder, tuberculosis, or recent traveling to other countries. Extensive laboratory evaluation revealed elevated NT-proB-type Natriuertic Peptide (1113.5 pg/ml; normal < 100 pg/ml) and C-reactive protein (1.21 mg/ dL, normal < 0.5 mg/dL), but normal findings of complete blood count, basic chemistry profile, troponin-I, sedimentation rate, antinuclear antibody, antimitochondrial antibody, serum calcium/phosphate level, vitamin D level, and thyroid and parathyroid hormone levels. His echocardiography showed: (1) normal LV systolic function with calcification in the LV septum, mid free wall and mitral valve annulus (Figure 4) and normal right ventricular (RV) size and systolic function; (2) moderate bi-atrial enlargement with mild mitral regurgitation, moderate tricuspid regurgitation; and (3) severe pulmonary hypertension and LV diastolic dysfunction. Cardiac catheterization demonstrated: (1) no angiographic evidence of coronary artery disease; (2) moderately severe pulmonary hypertension with pulmonary artery systolic pressure of 67 mmHg; (3) elevated pulmonary capillary wedge pressure, measured at 28 mmHg; (4) elevated LV end-diastolic pressure, measured 29 mmHg; and (5) no evidence of ventricular interdependence on simultaneous LV/RV pressure measurement.
The final diagnosis was congestive diastolic heart failure due to massive LV myocardial calcification of unknown etiology. He was treated with aggressive diuresis and other guideline-driven medical therapy successfully with marked improvement of his dyspnea on exertion during follow-up.