Abstract
The pathophysiology of myocardial calcifications is variable and may have potentially fatal consequences if undiagnosed and untreated.
A 36-year-old woman with systemic secondary amyloidosis and end-stage renal disease undergoing dialysis presented in septic shock. Echocardiography demonstrated mildly depressed left ventricular ejection fraction (40%) with basal-to-mid hypokinesis and preserved apical contractility. A chest CT scan revealed biventricular myocardial calcification, absent from imaging results obtained 28 days prior (Fig 1). She developed pulseless electrical activity resulting in death. Cardiac autopsy noted contraction band necrosis with minimal endocardial amyloid and patchy myocardial calcification (Fig 2). Dermal and soft-tissue calcification were also present, despite normocalcemia and hypophosphatemia.
Figure 1:
A, Axial chest CT scans in a 36-year-old woman demonstrate development of diffuse biventricular myocardial calcification from patient’s last known normal CT scan at an outside hospital (day 0) to presentation at our institution 28 days later (day 28). Calcifications were additionally observed in presternal and infrascapular soft tissue, left liver lobe, and in patchy areas within left lower and posterior right upper lobes of lungs. B, Three-dimensional cinematic rendering of patient’s heart on day 28 on a noncontrast CT image with windowing highlighting the extent of myocardial calcification.
Figure 2:
Photomicrograph of the myocardium. A, High-power image of myocardium demonstrates diffuse calcification (arrows). (hematoxylin-eosin stain; magnification, x200.) B, Image obtained with a special stain for calcium highlights numerous areas of calcification, which appear black in pale pink background (arrows). (von Kossa calcium stain; magnification, x200.)
Myocardial calcification is usually found incidentally following an insult (eg, infarction, sepsis, irradiation) or in the setting of biochemical abnormalities with metastatic calcification (eg, hyperuremia, chronic hemodialysis, hyperparathyroidism) (1,2). The pathophysiology in this case is uncertain, but the phenomenon is clinically important due to potential partial reversibility with treatment of the underlying cause (3,4). Left untreated, patients may develop irreversible myocardial damage, leading to arrhythmias, heart failure, and death.
Footnotes
Disclosures of Conflicts of Interest: J.Y.H. disclosed no relevant relationships. R.F. disclosed no relevant relationships. J.J. disclosed no relevant relationships.
References
- 1.Nance JW Jr, Crane GM, Halushka MK, Fishman EK, Zimmerman SL. Myocardial calcifications: pathophysiology, etiologies, differential diagnoses, and imaging findings. J Cardiovasc Comput Tomogr 2015;9(1):58–67. [DOI] [PubMed] [Google Scholar]
- 2.Woodhouse MA, Burston J. Metastatic calcification of the myocardium. J Pathol 1969;97(4):733–736. [DOI] [PubMed] [Google Scholar]
- 3.Bower G, Ashrafian H, Cappelletti S, et al. A proposed role for sepsis in the pathogenesis of myocardial calcification. Acta Cardiol 2017;72(3):249–255. [DOI] [PubMed] [Google Scholar]
- 4.Terman DS, Alfrey AC, Hammond WS, Donndelinger T, Ogden DA, Holmes JH. Cardiac calcification in uremia. A clinical, biochemical and pathologic study. Am J Med 1971;50(6):744–755. [DOI] [PubMed] [Google Scholar]