Rapid regression of marked left ventricular septal hypertrophy following immunosuppressive therapy in cardiac sarcoidosis

Abstract

A 46-year-old man presented with asymmetric remarkable left ventricular (LV) septal hypertrophy (maximal wall thickness: 24 mm) and complete atrio-ventricular block. Systemic sarcoidosis with a cardiac lesion was diagnosed by a supraclavicular lymph node biopsy. Following pacemaker implantation, the patient received immunosuppressive therapy with corticosteroids and methotrexate. One week after starting treatment, echocardiography and cardiac magnetic resonance (CMR) imaging showed reduced LV septal hypertrophy. At the 6-month follow-up, further thinning of the basal septal wall and enlargement of the LV with a decreased ejection fraction were observed, despite resolution of abnormal uptake in ¹⁸F-fluorodeoxyglucose positron emission tomography. A reduction in T2 values on CMR indicated that the initial hypertrophy was associated with edematous and inflammatory changes. Our findings suggest that there were heterogeneous lesions in the myocardium, such as edematous lesions responsive to immunosuppressive therapy and fibrotic lesions progressing to LV wall thinning.

Learning objective

Cardiac sarcoidosis with asymmetrical and remarkable hypertrophy is rare in the clinical setting, and using multimodality imaging in addition to histological findings is necessary for diagnosing this condition. Multimodality imaging including echocardiography and cardiac magnetic resonance are useful for evaluating myocardial characterization and confirming remarkable changes in left ventricular wall thickness and dysfunction after immunosuppression therapy.