(479) When Lightning Strikes Twice! An Interesting Case of Recurrent Cardiac Sarcoidosis in a Transplanted Heart

• Published in: The Journal of heart and lung transplantation Vol. 42; no. 4; p. S219
• Main Authors: Sharma, V., Duque, E. Ruiz, Mansour, S., Firchau, D., Yumul, I., Cadaret, L., Viray, M.
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01-04-2023
• ISSN: 1053-2498; 1557-3117
• DOI: 10.1016/j.healun.2023.02.494

Cardiac Sarcoidosis (CS) is a chronic granulomatous disease that can recur in transplanted hearts. There are currently no standardized guidelines for management of CS in heart transplant recipients.
A 58-year-old male with end-stage NICM (presumed chemotherapy-related cardiotoxicity) presented with shortness of breath, and persistent low flow alarms on his HVAD. His past medical history was significant for leukemia (treated with doxorubicin), non-sustained VT s/p ICD and HVAD as bridge-to-transplant (2016). RHC showed evidence of cardiogenic shock. He was upgraded to UNOS Status 2 and underwent orthotropic heart transplant (OHT) on 7/30/21. He received standard induction (Basiliximab) and maintenance (Tacrolimus, MMF, prednisone) immunosuppression regimen. Unexpectedly, his explanted heart was noted to have non-necrotizing granulomatous myocarditis consistent with CS. He was switched from MMF to Sirolimus at 7 months post-OHT after TTE showed normal biventricular function and surveillance biopsy revealed 0R, AMR0. He was weaned off prednisone at 8 months. Subsequent cardiac biopsy showed recurrence of non-necrotizing granulomas with focal infiltrates (Fig A), suggesting recurrence of CS and mild acute cellular rejection (1R, AMR0). Patient remained asymptomatic with normal biventricular function. He was restarted on high dose prednisone and switched from Sirolimus to MMF. Tacrolimus goal was kept at 8-10. Five months following CS recurrence, cardiac PET showed no evidence of CS on Tacrolimus, MMF and low-dose prednisone.
Recurrent CS in a transplanted heart is rare and usually manifests itself once immunosuppression is weaned. While there are no standard guidelines for its management, standard dose MMF plus low-dose prednisone in addition to Tacrolimus was effective in preventing further recurrence in our patient. Routine monitoring for recurrence is important for early detection and prevention of life-threatening complications.