**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Artur Schneider, Mayo Clinic, Jacksonville FL

**Main Body of the Abstract**
Amyloidosis is a systemic process characterized by extracellular deposition of insoluble amyloid fibrils, and cardiac amyloidosis results from myocardial fibril deposition. Transthyretin amyloidosis results from misfolded transthyretin precursor protein. The presence of advanced heart failure in cardiac amyloidosis is associated with worse prognosis. Orthotopic heart transplantation (HT) remains the definitive treatment, but patients can still develop extra-cardiac manifestations post HT. We present a patient with wild-type transthyretin cardiac amyloidosis (ATTRwt) who developed skull base amyloidosis resulting in trigeminal nerve compression post HT.
The patient is a 73-year-old male with ATTRwt cardiac amyloidosis who underwent HT two years prior and presented to the Emergency Department with right sided facial pain. Maxillofacial computed tomography and magnetic resonance imaging of the brain showed an infiltrative process spreading across the skull base, including the trigeminal cavity. Endoscopic endonasal biopsy was obtained from the left skull base and was consistent with amyloidosis, showing apple-green briefringence on Congo red stain. Liquid chromatography tandem mass spectrometry was consistent with ATTRwt amyloidosis. He underwent balloon decompression of his right trigeminal nerve to alleviate symptoms, but his retractable severe facial pain returned, complicated by nausea and poor oral intake, and he ultimately passed away.
Althouh HT remains the best option for end stage heart failure associated with cardiac amyloidosis, there remains concern for de novo or worsening of extracardiac manifestations post transplant. Further studies are needed regarding the use of disease targeted therapy to prevent progression of extracardiac manifestations in transthyretin amyloidosis post HT



Skull Base Amyloidosis post Heart Transplantation in a Patient with Wild Type Transthyretin Cardiac Amyloidosis PB67 (#201)

We invite you to attend the XIX International Symposium on Amyloidosis to be held in Rochester, MN.

This symposium is organized as a hybrid meeting (with the focus on in-person) held from Sunday, May 26 through Thursday, May 30, 2024.

Together with the ISA Board and members of Mayo Clinic, a very attractive scientific hybrid program is being planned. The program will cover a variety of types of systemic amyloidosis: ATTR (hereditary and wild-type), AL (light-chain), AA and less frequent forms. There will be key lectures, specific symposia including those company sponsored, oral presentations and poster sessions of accepted abstracts.

COURSE DESCRIPTION
This program will provide a comprehensive review of state-of-the-art education in systemic amyloidosis: basic science, pathophysiology, diagnosis, current and emerging treatments. Scientific presentations will include competitive oral and poster abstracts. The rapid evolution of scientific discovery and expanding treatment options has created tremendous interest in the field and this meeting will fill and important gap in educating scientists and clinicians.