Korean J Transplant.  2022 Nov;36(Supple 1):S363. 10.4285/ATW2022.F-5028.

Simultaneous heart and kidney transplantation in middle-aged women with cardiac amyloidosis

• Bak M
• Choi JO
• Kim D
• Jeon ES

• KMID: 2535241
• DOI: http://doi.org/10.4285/ATW2022.F-5028

Abstract

This is the story of a patient with cardiac amyloidosis who underwent heart transplant. A 56-years-old female underlying dia-betes mellitus visited our clinic with dyspnea and was admitted for further evaluation. On electrocardiogram, junctional rhythm and paroxysmal atrial fibrillation (AF) were shown, and NT-proBNP was over 2,000 pg/mL. The free light chain ratio was 93, and chronic kidney disease (CKD) stage 3 with microalbuminuria was confirmed. On cardiac echocardiography, both ventricular walls were thickened and a restrictive pattern of diastolic dysfunction was observed. Bone marrow biopsy showed 30% of plasma cells. During the hospital stay, permanent pacemaker was inserted due to symptomatic sinus pause. Neurological examination showed moderate autonomic dysfunction and mild carpal tunnel syndrome. Under the diagnosis of multiple myeloma with car-diac amyloidosis Mayo stage 4, chemotherapy was started. However, diastolic heart failure did not improve and the burden of AF became 100% during 2 years. Kidney function also progressed to CKD stage 4 due to anticancer drug toxicity and cardiorenal syndrome. Although a complete hematologic response was shown after third chemotherapy, the patient's quality of life declined sharply due to repeated hospitalization. The final neurological examination showed no orthostatic hypotension, and there were no findings suggestive of gastrointestinal amyloidosis. Heart and kidney transplantation was considered because there were no major disorders in other organ. After central extracorporeal membrane oxygenation, heart and kidney cotransplantation was performed. After 2 weeks, the patient complained of sudden abdominal pain, and duodenal perforation was diagnosed, and emergency primary repair was performed. But, next day, gastrojejunostomy was performed due to failure of primary repair. After that, the patient went through antibiotic treatment and rehabilitation for 2 months, and discharged in a state of being able to eat. Currently, the patient is maintaining a normal life without rejection at 8 months of transplantation.