End-stage heart failure and heart transplant in cardiac sarcoidosis: a case series.

Background: Diagnosing cardiac sarcoidosis (CS) is challenging. Immunosuppressive therapies are less effective in end-stage disease, and often heart transplant (HT) is the only available option. We present a series of advanced CS cases, requiring HT, along with a review of the literature evidence in this field.

Case Summary: Case 1: a 49-year-old man initially suspected of having arrhythmogenic cardiomyopathy (ACM) presented with heart failure (HF) and recurrent ventricular arrhythmias. The rapid clinical deterioration raised suspicion of an inflammatory aetiology, which was confirmed through endomyocardial biopsy, diagnosing CS. Despite immunosuppressive therapy, HT was required. Case 2: a 36-year-old woman presented with high-grade atrioventricular block and dilated cardiomyopathy (DCM), initially diagnosed as idiopathic. Due to worsening HF, she required HT. The pathological examination of the explanted heart revealed CS. Chronic subclinical antibody-mediated rejection was observed after HT. Case 3: a 44-year-old man presented with syncope and imaging suggesting ACM. He was referred for HT due to high ventricular arrhythmic burden. Cardiac sarcoidosis diagnosis was suspected due to pulmonary involvement and then confirmed on post-explant pathological exam. Post-HT pulmonary and cutaneous sarcoidosis reactivation were observed. Case 4: a 43-year-old man was diagnosed with pulmonary sarcoidosis after lung biopsy. Progression towards DCM was observed despite immunosuppressive therapy. Post-HT was characterized by multiple episodes of graft rejection.

Discussion: This case series provides insights into mid- and long-term outcomes after HT for CS, highlighting the need for careful management of immunosuppression in these patients, balancing the adverse effects of chronic immunosuppression with the prevention of rejection and sarcoidosis recurrence.