Porcelain heart—an unusual case of massive myocardial calcification

Abstract Background Myocardial calcification is an unusual entity and an infrequent imaging finding. Although localized myocardial calcifications are prevalent after myocardial infarction, extensive myocardial calcification is uncommon. Historically, two main pathologic mechanisms have been recognized: metastatic and dystrophic . Here, we report a rare case of extensive dystrophic myocardial calcification in an adolescent girl with primary immunodeficiency syndrome, where imaging evidence and clinical history helped confirm the diagnosis. Case presentation. A 15-year-old adolescent girl, who was clinically diagnosed with chronic granulomatous disease, presented with respiratory tract infection and left-sided chest discomfort. She was found to have symptomatic bradycardia, and an ECG revealed a complete heart block. The patient was admitted to the Cardiac Care Unit and then referred for a cardiac MRI to identify a potential cause for the complete heart block. MR imaging showed the following findings: Multiple T2 hypointensities were noted in the interventricular septum and the apex indicating calcifications, along with patchy mid-myocardial enhancement in the septum, indicating fibrosis. Chest X-ray and CT also demonstrated extensive myocardial calcifications. A detailed retrospective clinical history revealed several hospitalizations for sepsis in her childhood. Thus, based on clinical history, imaging, and other laboratory parameters, a diagnosis of sepsis-related myocardial calcification was made. Conclusion In conclusion, we diagnosed a rare case of sepsis-related myocardial calcification in its early phases, with progressive myocardial calcification and ejection fraction decline over time. Only a few cases of myocardial calcifications have been documented in the literature, and only a few have been evaluated using cardiac MR. Serially assessing CT and echocardiography from the onset of sepsis allows for early detection and proper therapy, potentially improving clinical outcomes. Patients with sepsis-related myocardial calcification, even if their ejection fraction is preserved, should be closely monitored for further calcium deposition and consequent left ventricle systolic dysfunction.