**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.**
Mazen Hanna, MD, Cleveland Clinic, Cleveland, OH

**Main Body of the Abstract**
Case Report:
An African American male with p.Val142Ile (Val122Ile) variant transthyretin cardiac amyloidosis who progressed to end stage heart failure received an isolated heart transplant without concomitant liver transplant in 2008 at the age of 65 years. At 8 years post-transplant, he developed two scleral deposits removed with pathology showing amyloid. Starting about 12 years post-transplant, he began to develop progressive and disabling peripheral and autonomic neuropathy that greatly limited his mobility and led to issues of orthostatic hypotension.
In 2023, 15 years after heart transplant at the age of 80 years, he presented with progressive and severe macroglossia affecting his ability to eat. A tongue biopsy was performed (see figure) which showed amyloid deposition in the stroma as confirmed by Congo Red stain . Typing with immunohistochemistry was consistent with transthyretin type. He was given a soft food diet until he died 9 months later.  Of note, 3 months prior to his death he underwent an endomyocardial biopsy due to some changes on echocardiogram which showed no evidence of recurrent amyloid deposition.
Conclusion:  Long term survival in this patient with p.Val142Ile transthyretin amyloidosis after isolated heart transplantation shows that extracardiac progression is slow and progressive. Although macroglossia has been described rarely in transthyretin amyloidosis, to our knowledge this is the first case described in the p.Val142Ile variant and after heart transplantation. Disease modifying therapies in the current era should decrease extracardiac progression for patients that undergo heart transplantation for hereditary transthyretin amyloidosis.



**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.**
Mazen Hanna, MD, Cleveland Clinic, Cleveland, OH

**Main Body of the Abstract**
**Case Report:**
An African American male with p.Val142Ile (Val122Ile) variant transthyretin cardiac amyloidosis who progressed to end stage heart failure received an isolated heart transplant without concomitant liver transplant in 2008 at the age of 65 years. At 8 years post-transplant, he developed two scleral deposits removed with pathology showing amyloid. Starting about 12 years post-transplant, he began to develop progressive and disabling peripheral and autonomic neuropathy that greatly limited his mobility and led to issues of orthostatic hypotension.
In 2023, 15 years after heart transplant at the age of 80 years, he presented with progressive and severe macroglossia affecting his ability to eat. A tongue biopsy was performed (see figure) which showed amyloid deposition in the stroma as confirmed by Congo Red stain . Typing with immunohistochemistry was consistent with transthyretin type. He was given a soft food diet until he died 9 months later.  Of note, 3 months prior to his death he underwent an endomyocardial biopsy due to some changes on echocardiogram which showed no evidence of recurrent amyloid deposition.
**Conclusion:**  Long term survival in this patient with p.Val142Ile transthyretin amyloidosis after isolated heart transplantation shows that extracardiac progression is slow and progressive. Although macroglossia has been described rarely in transthyretin amyloidosis, to our knowledge this is the first case described in the p.Val142Ile variant and after heart transplantation. Disease modifying therapies in the current era should decrease extracardiac progression for patients that undergo heart transplantation for hereditary transthyretin amyloidosis.



Severe Macroglossia in a Patient with p.Val142Ile Hereditary Transthyretin Amyloidosis Manifesting 15 years After Heart Transplantation PB155 (#568)

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.