Heart and Blood Diseases

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Blood and Lymph Conditions

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Cardiomyopathies

Amyloidosis

Immunoglobulin Light-chain Amyloidosis

Heart Diseases

Proteostasis Deficiencies

Metabolic Diseases

Neoplasms, Plasma Cell

Neoplasms by Histologic Type

Lymphoproliferative Disorders

Immunoproliferative Disorders

Paraproteinemias

AL Amyloidosis

Participants in this cohort are individuals undergoing clinically indicated cardiac device implantation, such as pacemakers, ICDs, ILRs, or CRT-D/Ps. During the standard implantation procedure, chest wall fat tissue typically excised to create space for the device is collected for histopathological analysis to identify amyloid deposits. This study involves no additional intervention beyond the routine clinical care received by the patients.

As part of routine cardiac device implantation, chest wall fat tissue is collected for histopathological analysis. This tissue, which is typically discarded, will be used to identify amyloid deposits in high-risk cardiac patients. No additional surgical intervention is performed beyond the standard procedure for device implantation.

Chest Wall Fat Tissue Collection

Vasvi Singh, MD

Elizabeth Fulks, MS

This single-practice prospective cohort study aims to enhance the diagnosis of cardiac amyloidosis in high-risk patients undergoing standard cardiac device implantation. By analyzing chest wall fat tissue, which is usually discarded, we aim to determine the diagnostic yield of such biopsies for amyloidosis and to develop a predictive screening model based on clinical, lab, and imaging data. The study, running from December 2023 to December 2024, expects to enroll 100 patients and may provide a new, non-invasive diagnostic avenue for this condition.

The study targets a key gap in cardiac amyloidosis diagnosis by systematically evaluating the histopathological incidence of the disease using chest wall fat pad biopsies-tissue that is typically discarded during the implantation of cardiac devices like pacemakers, ICDs, and CRT-D/Ps. Standard surgical procedures are adhered to, ensuring minimal additional risk to patients. The collected tissue samples are analyzed by the HCA pathology laboratory to detect amyloid deposits, thereby potentially identifying amyloidosis in a non-invasive manner. In addition to the primary endpoint of histopathological diagnosis, the study retrospectively aims to validate a predictive model that incorporates a wide range of data to streamline the identification of patients at high risk for cardiac amyloidosis. Strict measures are in place to protect patient confidentiality and data security. By potentially improving diagnostic efficiency, this research could contribute to earlier detection and treatment strategies, thus improving patient outcomes for those at high risk of this life-threatening condition.

The biospecimens consist of chest wall fat tissue samples collected during cardiac device implantation procedures. This adipose tissue, typically excised to create space for the device, is preserved for histopathological analysis to detect amyloid deposits. The analysis of these samples is crucial for diagnosing cardiac amyloidosis in patients at high risk. The tissue will be handled and analyzed by the HCA pathology laboratory, following standard procedures to ensure the integrity of the samples and the accuracy of the diagnostic results.

Amyloidosis Incidence in High-Risk Cardiac Device Patients

Histopathological Incidence of Amyloidosis in High-Risk Patients Undergoing Cardiac Device Implantation

At present, the specifics of a data-sharing plan for individual participant data (IPD) from this study are under consideration. The study team intends to protect patient confidentiality and data integrity and will comply with all relevant regulations and guidelines for data sharing. Once a plan has been established, details about the nature of the data to be shared, the conditions for access, and the timing for data release will be provided.

The primary outcome measure is the histopathologic diagnosis of amyloidosis in chest wall fat tissue removed during cardiac device implantation

Retrospective development and validation of a predictive model using pre-procedural clinical, lab, and imaging data to identify patients at high risk for cardiac amyloidosis

Midwest Heart & Vascular Specialists

Anti-Inflammatory Agents

Antineoplastic Agents

Antiemetics

Gastrointestinal Agents

All Drugs and Chemicals

Dexamethasone

Bortezomib

Pomalidomide

Dexamethasone acetate

Glucocorticoids

Hormones

Hormone Antagonists

Antineoplastic Agents, Hormonal

Immunologic Factors

Angiogenesis Inhibitors

Growth Inhibitors

BPd regimen (Bortezomib, pomalidomide, and dexamethasone) will be applied every 28 days until progression of disease, unacceptable toxicity or subsequent therapy, for a maximum of six cycles.

4mg per day taken orally on 1-21 of repeated 28-day cycles

Yang Liu, Dr

Yang Liu

Jin Lu

This is an open-label, multicenter, Phase 2 study in subjects with newly diagnosed systemic light chain (AL) amyloidosis. Approximately 40 subjects will receive therapy with bortezomib, pomalidomide, and dexamethasone. The primary outcome is hematologic very good partial response and complete response rate at 6 months.

Proteasome inhibitor and immunmodulators have synergistic effect for plasma cell dyscrasia. Due to the fact that more than 70-80% of patients with amyloidosis have renal involvement, the application of lenalidomide is limited. Thus, the investigators designed this open-label, multicenter, phase 2 study for newly diagnosed or previously treated systemic AL amyloidosis with bortezomib, pomalidomide and dexamethasone regimen.

Bortezomib, Pomalidomide, Dexamethasone For Systemic AL Amyloidosis

A Study of Bortezomib, Pomalidomide, Dexamethasone in Patients With Systemic AL Amyloidosis

Hematologic very good partial response plus complete response rate at 6 months

Overall Hematologic partial response, very good partial response rate and complete response at 6 months

At least one organ response (cardiac response, renal response, liver response) at 6 months

Estimated Progression free survival at 2 years

Treatment-related adverse events up to 6 months

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Amyloidosis Incidence in High-Risk Cardiac Device Patients	NCT06186167	Amyloid Cardiac Amyloid... Amyloidosis Car... Systemic Amyloi... AL Amyloidosis Infiltrative Ca... ATTR Amyloidosi...	Chest Wall Fat ...	40 Years -	Midwest Heart & Vascular Specialists
Bortezomib, Pomalidomide, Dexamethasone For Systemic AL Amyloidosis	NCT06342466	Systemic Amyloi...	Bortezomib Pomalidomide Dexamethasone	18 Years - 90 Years	Peking University People's Hospital
Amyloidosis Incidence in High-Risk Cardiac Device Patients	NCT06186167	Amyloid Cardiac Amyloid... Amyloidosis Car... Systemic Amyloi... AL Amyloidosis Infiltrative Ca... ATTR Amyloidosi...	Chest Wall Fat ...	40 Years -	Midwest Heart & Vascular Specialists
Amyloidosis Incidence in High-Risk Cardiac Device Patients	NCT06186167	Amyloid Cardiac Amyloid... Amyloidosis Car... Systemic Amyloi... AL Amyloidosis Infiltrative Ca... ATTR Amyloidosi...	Chest Wall Fat ...	40 Years -	Midwest Heart & Vascular Specialists

Spots Global Cancer Trial Database for systemic amyloidosis