(434) An Alarming Surprise

• Published in: The Journal of heart and lung transplantation Vol. 42; no. 4; p. S202
• Main Authors: Shahandeh, N., David, S., King, M., Smith, J., Fishbein, M., Biniwale, R., Nsair, A., Kamath, M.
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01-04-2023
• ISSN: 1053-2498; 1557-3117
• DOI: 10.1016/j.healun.2023.02.449

Systemic immunoglobulin light-chain (AL) amyloidosis is a plasma cell disorder that can cause cardiomyopathy due to the extracellular deposition of amyloid in the heart. When left untreated, median survival is 6 months. We report a case of AL amyloidosis diagnosed on pathology of the explanted heart after transplantation in a patient who presented in cardiogenic shock.
A 54-year-old woman with a recent diagnosis of nonischemic cardiomyopathy attributed to COVID-19 vaccine-associated myocarditis presented to a community hospital in cardiogenic shock. Right heart catheterization revealed elevated filling pressures (right atrium 19 mmHg, pulmonary capillary wedge 43 mmHg) and reduced cardiac index (2.1 L/min/m2). Echocardiography showed normal left ventricular size and wall thickness with severely reduced systolic function (ejection fraction 20%), findings that were unchanged from a study performed 3 months prior. The patient was started on inotropic support with milrinone and dobutamine and transferred to our institution. Shortly after transfer, she became unstable and we instituted mechanical circulatory support with venoarterial extracorporeal membrane oxygenation. Expedited heart transplant evaluation was completed and she was listed at status 1 priority. She was discharged on post-operative day 10 after uncomplicated heart transplantation. Pathology of the explanted heart revealed kappa AL amyloidosis. The patient was referred to oncology and began daratumumab monotherapy 3 months post-transplant with improvement in the light chain burden. Surveillance endomyocardial biopsies have not shown evidence of amyloidosis and the patient is doing well clinically.
Early diagnosis and treatment of AL amyloidosis are vital to prevent progression of cardiomyopathy and may obviate the need for heart transplantation. For those who undergo heart transplantation, continued treatment directed at the plasma cell clone with chemotherapy or stem cell transplantation is essential to ensuring long-term success of the allograft.