572 intramyocardial calcification in apical hypertrophic cardiomyopathy assessed using multimodality imaging: our case series

Abstract Introduction Apical Hypertrophic Cardiomyopathy (ApHCM) characterized by persistent diastolic apical contraction results in dynamic apical small-vessel obstruction with microvascular ischemia. Of note, endomyocardial fibrosis (EMF) and calcification are described only in few patients. Case presentation We report 5 cases of ApHCM with apical intramyocardial calcification. They presented characteristic ECG pattern and fibrosis at echocardiogram. All patients presented a preserved ejection fraction (EF), except for one patient with mild reduced EF. Global longitudinal strain was reduced in 3 patients. Diastolic dysfunction was evidenced in 3 patients. Right ventricle involvement was detected in one patient only.On cardiac magnetic resonance, a superficial hypo-intense component, compatible with calcium and a deep layer featured by late gadolinium enhancement (LGE) related to fibrotic tissue, were revealed. LGE was present in all of patients in the apex. One patient presented an apical aneurysm, with high ESC-SCD risk score and ICD implantation. Conclusion EMF pathologic hallmarks were the endocardium and myocardium scarring, evolving to dystrophic calcification. In clinical practice, only a minority of ApHCM patients develops EMF and calcifications. Our clinical series is the largest one in literature. Analyzing patients’ history, a microvascular inflammatory trigger was evident in all of them, particularly severe chronic kidney disease, diabetes, high degree obesity, malaria infection, peripheral microangiopathy and form of thalassemia. This series could demonstrate the pathophysiological relation between apical fibrosis, calcification and microvascular ischemia due to hypertrophy and inflammatory conditions coexistence. A broader case series could evaluate any correlation with their long-term outcome and management strategies.