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The purpose of this study to assess the longitudinal changes in left atrial strain and supraventricular arrhythmia burden after chemotherapeutic strategies in cardiac light chain amyloidosis.
Light chain (AL) amyloidosis is a disease characterized by localized or systemic accumulation of amyloid fibrils in tissues caused by abnormal folding of light chain immunoglobulins and affecting organ functions. Due to the accumulation of abnormal folding immunoglobulins in the cardiac conduction system, arrhythmia susceptibility has been reported to increase significantly. Atrioventricular blocks and supraventricular arrhythmia are mainly detected among the most common arrhythmia. While these developing supraventricular arrhythmias and atrioventricular blocks in non-amyloidosis diseases result in increased mortality and morbidity, there is insufficient data on cardiac AL amyloidosis treated with chemotherapy. For this reason, this study was planned, and the purpose of this study is to assess the longitudinal changes in left atrial strain and supraventricular arrhythmia burden after chemotherapeutic strategies in cardiac light chain amyloidosis.
After being informed about the study, it is planned to recruit and follow up AL-Amyloidosis patients with cardiac involvement who are currently receiving/planned chemotherapy followed by hematology or who are planned for bone marrow transplantation within one year after obtaining written consent from the patients.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Light Chain Cardiac Amyloidosis | Participants with light chain cardiac amyloidosis actively receiving chemotherapy or undergoing autologous bone marrow transplant |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Left Atrial Strain Imaging | Diagnostic Test | Two-dimensional, color Doppler, spectral Doppler recordings will be taken from parasternal, apical, subcostal, and modified sections using a Philips Epiq CVx echocardiography device and X5-1 probe. Conventional parameters, additional parameters, and strain analyzes will be performed with speckle tracking echocardiography from these recordings. |
| Measure | Description | Time Frame |
|---|---|---|
| The prognostic significance of longitudinal changes in left atrial strain and supraventricular arrhythmia burden after chemotherapy in cardiac light chain amyloidosis | In this study, the effects of treatment strategies on left atrial strain imaging and supraventricular arrhythmia burden and its relationship with mortality and morbidity will be investigated in patients with AL-amyloidosis, who are still under treatment, and newly diagnosed with AL-amyloidosis. | Baseline, and 1st, 3rd, 6th, 12th Month follow-up |
| Measure | Description | Time Frame |
|---|---|---|
| Evaluation of the effects of treatment strategies on arrhythmia burden | By obtaining the serial ECG records, arrhythmia burden will be recorded/ | Baseline, and 1st, 3rd, 6th, 12th Month follow-up |
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Inclusion Criteria:
Exclusion Criteria:
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Patients aged 18-90 years with cardiac primary light chain amyloidosis or due to multiple myeloma who are newly diagnosed, currently receiving chemotherapeutic treatment, or are scheduled for bone marrow transplantation will be recruited.
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| Name | Affiliation | Role |
|---|---|---|
| Burçak Kılıçkıran Avcı, Assoc Prof. | Istanbul University - Cerrahpasa | Study Chair |
| Deniz Mutlu, MD | Istanbul University - Cerrahpasa | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Istanbul University-Cerrahpasa | Istanbul | 34098 | Turkey (Türkiye) |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 34396169 | Result | Witteles RM, Liedtke M. AL Amyloidosis for the Cardiologist and Oncologist: Epidemiology, Diagnosis, and Management. JACC CardioOncol. 2019 Sep 24;1(1):117-130. doi: 10.1016/j.jaccao.2019.08.002. eCollection 2019 Sep. | |
| 29596561 | Result | Badano LP, Kolias TJ, Muraru D, Abraham TP, Aurigemma G, Edvardsen T, D'Hooge J, Donal E, Fraser AG, Marwick T, Mertens L, Popescu BA, Sengupta PP, Lancellotti P, Thomas JD, Voigt JU; Industry representatives; Reviewers: This document was reviewed by members of the 2016-2018 EACVI Scientific Documents Committee. Standardization of left atrial, right ventricular, and right atrial deformation imaging using two-dimensional speckle tracking echocardiography: a consensus document of the EACVI/ASE/Industry Task Force to standardize deformation imaging. Eur Heart J Cardiovasc Imaging. 2018 Jun 1;19(6):591-600. doi: 10.1093/ehjci/jey042. |
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|
|
| Electrocardiography | Diagnostic Test | Electrocardiography will be taken at baseline and the follow-up periods |
|
|
| 35323616 | Result | Inoue K, Kawakami H, Akazawa Y, Higashi H, Higaki T, Yamaguchi O. Echocardiographic Assessment of Atrial Function: From Basic Mechanics to Specific Cardiac Diseases. J Cardiovasc Dev Dis. 2022 Feb 27;9(3):68. doi: 10.3390/jcdd9030068. |
| 34729522 | Result | Hartnett J, Jaber W, Maurer M, Sperry B, Hanna M, Collier P, Patel DR, Wazni OM, Donnellan E. Electrophysiological Manifestations of Cardiac Amyloidosis: JACC: CardioOncology State-of-the-Art Review. JACC CardioOncol. 2021 Oct 19;3(4):506-515. doi: 10.1016/j.jaccao.2021.07.010. eCollection 2021 Oct. |
| ID | Term |
|---|---|
| D028227 | Amyloid Neuropathies, Familial |
| D006333 | Heart Failure |
| D001145 | Arrhythmias, Cardiac |
| ID | Term |
|---|---|
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D009422 | Nervous System Diseases |
| D017772 | Amyloid Neuropathies |
| D010523 | Peripheral Nervous System Diseases |
| D009468 | Neuromuscular Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D028226 | Amyloidosis, Familial |
| D008661 | Metabolism, Inborn Errors |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D000686 | Amyloidosis |
| D057165 | Proteostasis Deficiencies |
| D006331 | Heart Diseases |
| D002318 | Cardiovascular Diseases |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
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| ID | Term |
|---|---|
| D004562 | Electrocardiography |
| ID | Term |
|---|---|
| D006334 | Heart Function Tests |
| D003935 | Diagnostic Techniques, Cardiovascular |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D004568 | Electrodiagnosis |
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