Concurrent Transthyretin Cardiac Amyloidosis and Light Chain Amyloidosis of the Foregut

2020 ◽  
Vol 26 (10) ◽  
pp. S100
Author(s):  
Bryan E-Xin Tan ◽  
Samarthkumar Thakkar ◽  
Vishal Parikh
Keyword(s):  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Light Chain Amyloidosis

scholarly journals Diagnostic approach to light-chain cardiac amyloidosis and its differential diagnosis

2018 ◽  
Vol 49 (1) ◽  
pp. 9-14
Author(s):  
Monika Adamska ◽  
Anna Komosa ◽  
Tatiana Mularek ◽  
Joanna Rupa-Matysek ◽  
Lidia Gil
Keyword(s):  
Differential Diagnosis ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Diagnosis And Treatment ◽  
Diagnostic Approach ◽  
Al Amyloidosis ◽  
Delay In Diagnosis ◽  
Light Chain Amyloidosis ◽  
Novel Approaches

AbstractCardiac amyloidosis is a rare and often-misdiagnosed disorder. Among other forms of deposits affecting the heart, immunoglobulin-derived light-chain amyloidosis (AL amyloidosis) is the most serious form of the disease. Delay in diagnosis and treatment may have a major impact on the prognosis and outcomes of patients. This review focuses on the presentation of the disorder and current novel approaches to the diagnosis of cardiac involvement in AL amyloidosis.

Isolated cardiovascular involvement in light chain amyloidosis

2020 ◽  
Vol 13 (4) ◽  
pp. e233227
Author(s):  
Ahamed Shaheer Ahmed ◽  
Sampath Kumar ◽  
Gautam Sharma ◽  
Sudheer Arava
Keyword(s):  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Low Voltage ◽  
Systolic Dysfunction ◽  
Organ System ◽  
Lambda Light Chain ◽  
Text Book ◽  
Light Chain Amyloidosis ◽  
Amyloid Deposits ◽  
Cardiovascular Involvement

A 50-year-old woman presented with complaints of palpitations and breathlessness of 6 months’ duration. She was being treated elsewhere as a case of dilated cardiomyopathy. On evaluation she had racoon eyes, poor progression of R wave on ECG and low-voltage complexes in the limb leads. Echocardiography revealed biventricular hypertrophy, diastolic dysfunction and moderate systolic dysfunction. Cardiac MRI showed features suggestive of amyloidosis. Bone marrow biopsy revealed raised plasma cell count, and endomyocardial biopsy showed amyloid deposits in the myocardium. Free lambda light chain levels were elevated, even though serum and urine electrophoresis did not show any monoclonal band. In this ‘text book case of cardiac amyloidosis’, apart from cardiovascular system no other organ system was affected, which is uncommon in primary light chain amyloidosis. The patient was started on CyBorD (cyclophosphamide, bortezomib and dexamethasone) regimen.

scholarly journals 110 Sex-related differences in clinical presentation and outcome of patients with cardiac transthyretin amyloidosis and light chain amyloidosis

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Mattia Zampieri ◽  
Alessia Argirò ◽  
Marco Allinovi ◽  
Luigi Tassetti ◽  
Chiara Zocchi ◽  
...  
Keyword(s):  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Clinical Presentation ◽  
Nyha Class ◽  
Interventricular Septum ◽  
University Hospital ◽  
Wild Type ◽  
Transthyretin Amyloidosis ◽  
Light Chain Amyloidosis ◽  
Different Types

Abstract Aims We aimed to ascertain whether sex-related differences are relevant to the pathophysiology, presentation, and outcomes in different types of cardiac amyloidosis—a field still poorly investigated. Medical files from consecutive patients diagnosed with cardiac amyloidosis between 2000 and 2020, at Careggi University Hospital, were retrospectively evaluated. Methods and results Over this period, 259 patients (12% females) were diagnosed with wild type transthyretin amyloidosis (wtATTR), 52 (25% females) with hereditary transthyretin amyloidosis (hATTR) and 143 (47% females) with light chain amyloidosis (AL). Wt-ATTR women, compared to men, were significantly older at the time of diagnosis and showed higher National Amyloidosis Centre score, thicker normalized interventricular septum, higher diastolic dysfunction and worse right ventricular function. Females with hATTR and AL had lower normalized cardiac mass compared to men. Otherwise, bio-humoural parameters, NYHA class, and ECG characteristics were similar. No differences in outcome were observed with regard to sex. Conclusions In conclusion, we did not observe major differences in clinical expression related to sex in different types of cardiac amyloidosis: specifically, outcome was not affected. Nevertheless, women with wtATTR showed a worse profile at diagnosis and evidence suggesting a later recognition of disease compared to men, highlighting the need for a higher index of suspicion in female patients.

P29 Arrest of progression of cardiac amyloidosis after chemotherapy predicts favorable outcome in patients with light-chain amyloidosis

2020 ◽  
Vol 41 (Supplement_1) ◽  
Author(s):  
J Koyama ◽  
M Minamisawa ◽  
K Kuwahara
Keyword(s):  
Wall Thickness ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Left Ventricular ◽  
Favorable Outcome ◽  
Rank Test ◽  
Al Amyloidosis ◽  
Interval Change ◽  
Light Chain Amyloidosis

Abstract Funding Acknowledgements none Background Many studies demonstrated that cardiac involvement predicts poor prognosis in patients with systemic light-chain amyloidosis (AL). There is no data about the effect of the arrest of progression of cardiac amyloidosis on prognosis after chemotherapy. Hypothesis Arrest of progression of cardiac amyloidosis is associated with favorable outcome in patients with light-chain amyloidosis. Methods Among 126 consecutive patients with AL, we prospectively examined 94 patients serially after optimal therapy. The mean follow-up period was 1405 ± 1510 days (median value 734 days, inter quartile range 176-2343 days). Wall thickness was measured from left ventricular (LV) m-mode trace. We defined the cardiac involvement as mean LV wall thickness >12mm, and the regression or progression of cardiac amyloidosis as change in LV mean wall thickness >1mm. Results Among 94 patients with AL, 28 patients (30%) showed regression by definition above, 35 patients (37%) showed no interval change and 31 patients (33%) showed progression of cardiac amyloidosis. Survival analysis of 3 groups demonstrated that the regression and arrest of progression groups showed better outcome compared with the progression group (Log-rank test P < 0.0001). Conclusions The arrest of progression of cardiac amyloidosis predicts favorable outcome in patients with AL amyloidosis. Abstract P29 Figure. Kaplan-Meier Curve of 3 groups

scholarly journals Diagnosis and supportive therapeutic management of cardiac light chain amyloidosis—a cardiologist’s perspective

2021 ◽  
Author(s):  
Christina Binder ◽  
Franz Duca
Keyword(s):  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Therapeutic Management ◽  
Al Amyloidosis ◽  
Imaging Features ◽  
Transthyretin Amyloidosis ◽  
Light Chain Amyloidosis ◽  
Novel Treatments ◽  
Amyloid Deposits ◽  
Folded Proteins

SummaryCardiac amyloidosis is caused by deposition of abnormally folded proteins (amyloid). The most common forms of amyloidosis which present with cardiac involvement are light-chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). Even with novel treatments emerging, the prognosis of these patients remains poor once amyloid deposits in the heart. Therefore, knowledge on clinical and imaging features of cardiac amyloidosis is crucial to make an early diagnosis and improve patient outcomes. This article reviews the most important diagnostic findings of cardiac amyloidosis and gives an overview on the therapeutic management of these patients, including supportive-, device- and disease-specific drug therapies focusing on AL amyloidosis.

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