Cardiac amyloidosis is a progressive disease characterized by the buildup of amyloid fibrils in the extracellular space of the heart.It is divided in 2 main types,immunoglobulin light chain amyloidosis and transthyret...Cardiac amyloidosis is a progressive disease characterized by the buildup of amyloid fibrils in the extracellular space of the heart.It is divided in 2 main types,immunoglobulin light chain amyloidosis and transthyretin amyloidosis(ATTR),and ATTR amyloidosis is further divided in 2 subtypes,non-hereditary wild type ATTR and hereditary mutant variant amyloidosis.Incidence and prevalence of ATTR cardiac amyloidosis is increasing over the last years due to the improvements in diagnostic methods.Survival rates are improving due to the development of novel therapeutic strategies.Tafamidis is the only disease-modifying approved therapy in ATTR amyloidosis so far.However,the most recent advances in medical therapies have added more options with the potential to become part of the therapeutic armamentarium of the disease.Agents including acoramidis,eplontersen,vutrisiran,patisiran and anti-monoclonal antibody NI006 are being investigated on cardiac function in large,multicenter controlled trials which are expected to be completed within the next 2-3 years,providing promising results in patients with ATTR cardiac amyloidosis.However,further and ongoing research is required in order to improve diagnostic methods that could provide an early diagnosis,as well as survival and quality of life of these patients.展开更多
BACKGROUND Pulmonary hypertension is a rare cardiopulmonary disease,with an insidious onset that usually worsens rapidly.Amyloid light chain(AL)amyloidosis is a rare systemic disease caused by extracellular deposition...BACKGROUND Pulmonary hypertension is a rare cardiopulmonary disease,with an insidious onset that usually worsens rapidly.Amyloid light chain(AL)amyloidosis is a rare systemic disease caused by extracellular deposition of pathologic,insoluble,and proteinaceous fibrils in organs and tissues;however,it is difficult to diagnose given its varied and nonspecific symptoms.To date,rare cases of amyloidosis with pulmonary hypertension have been reported.Of note,the optimal treatments for cardiac amyloidosis complicated with pulmonary hypertension remain unclear.CASE SUMMARY We report a case of a 51-year-old woman who presented with progressively worsening dyspnea.Transthoracic echocardiography indicated severe pulmonary hypertension.Twenty-seven months after first admission,the patient returned with symptoms of progressive heart failure.A myocardial tissue sample stained with Congo red was positive,and the patient was ultimately diagnosed with AL amyloidosis with cardiac involvement.CONCLUSION Although pulmonary hypertension may be idiopathic,it is frequently associated with other conditions.In rare cases,pulmonary hypertension can be a complication of AL amyloidosis,which should be seriously considered in any adult presenting with nonspecific signs or symptoms of cardiac distress.展开更多
Purpose: Transthyretin cardiac amyloidosis (ATTR-CA) has been linked to many extra-cardiac manifestations including bilateral carpal tunnel syndrome (CTS). The aim of this study is to analyze patients with bilate...Purpose: Transthyretin cardiac amyloidosis (ATTR-CA) has been linked to many extra-cardiac manifestations including bilateral carpal tunnel syndrome (CTS). The aim of this study is to analyze patients with bilateral CTS to identify patients with high-risk features or “red flags” for ATTR-CA, identify if systematic screening was done for ATTR-CA and define opportunities for improved detection. Methods: Out of >5000 patients with bilateral CTS evaluated in a single tertiary care center in Southeast Michigan (2010-2016), we retrospectively studied a focused population of patients: men > 50 years and women > 60 years old with bilateral CTS and atrial fibrillation (n = 295). Baseline demographic, comorbidities, and electrocardiographic and echocardiographic findings were analyzed. A high-risk group suspicious for ATTR-CA was identified as patients with bilateral CTS, atrial fibrillation, and concomitant “red flags” including heart failure and left ventricular hypertrophy. Results: Out of 295 patients, 51.2% were female, 75.6% were White, and 22.4% were African American. Upon comparing the high-risk group (n = 67) with the remaining study population (n = 228), both diagnosis of ATTR-CA and mortality were higher among the high-risk group (7.5% vs 0.4% and 43.3% vs 24.6%, respectively, P = 0.003). Conclusions: A substantial number of bilateral CTS patients had additional “red flags” warranting formal evaluation for ATTR-CA;however, systematic evaluation for cardiac amyloidosis was not performed in many patients. This emphasizes that Multidisciplinary collaboration is needed to create a systematic workflow and to raise awareness amongst cardiologists and other physicians for suspecting ATTR-CA in bilateral CTS patients who have additional “red flags”.展开更多
Cardiac amyloidosis is a heterogeneous and challenging diagnostic disease with poor prognosis that is now being altered by introduction of new therapies.Echocardiography remains the first-line imaging tool, and when d...Cardiac amyloidosis is a heterogeneous and challenging diagnostic disease with poor prognosis that is now being altered by introduction of new therapies.Echocardiography remains the first-line imaging tool, and when disease is suspected on echocardiography, cardiac magnetic resonance imaging and nuclear imaging play critical roles in the non-invasive diagnosis and evaluation of cardiac amyloidosis. Advances in multi-modality cardiac imaging allowing earlier diagnosis and initiation of novel therapies have significantly improved the outcomes in these patients. Cardiac imaging also plays important roles in the risk stratification of patients presenting with cardiac amyloidosis. In the current review, we provide a clinical and imaging focused update, and importantly outline the imaging protocols, diagnostic and prognostic utility of multimodality cardiac imaging in the assessment of cardiac amyloidosis.展开更多
Introduction: Cardiac amyloidosis is a rare and under-diagnosed disease. The objective of this study was to collect cases of cardiac amyloidosis in patients hospitalized in the cardiology department of the Centre Hosp...Introduction: Cardiac amyloidosis is a rare and under-diagnosed disease. The objective of this study was to collect cases of cardiac amyloidosis in patients hospitalized in the cardiology department of the Centre Hospitalier Intercommunal Alençon-Mamers (CHICAM). Patients and Methods: This was a retrospective descriptive study of the records of patients diagnosed with cardiac amyloidosis. The study took place in the cardiology department of the CHICAM over 12 months (from January 1, 2020 to December 31, 2020). We included the files of patients in whom the diagnosis of cardiac amyloidosis was confirmed on the basis of clinical, echocardiographic, biological, scintigraphic and cardiac MRI evidence. Results: Seven patients were included. The mean age was 86.71 years. Six men for one woman. Most of the patients were in heart failure. There was one case of periorbital ecchymosis. Troponinemia was increased in three patients, NTproBNP was always high with a mean of 1000 ng/L. Cardiac echography showed a hyperbright septum in four cases, concentric hypertrophy of the left ventricle with a mean interventricular septum in diastole of 13.85 mm. Six cases were senile transthyretin amyloidosis (TTR), one case was mutated transthyretin amyloidosis (TTRm). Two cases of death were noted. Conclusion: Cardiac amyloidosis has a poor prognosis due to delayed diagnosis.展开更多
BACKGROUND Cardiac amyloidosis,a disease caused by the precipitation of amyloid proteins in the myocardial extracellular matrix has been historically difficult to diagnose due to lack of specific clinical manifestatio...BACKGROUND Cardiac amyloidosis,a disease caused by the precipitation of amyloid proteins in the myocardial extracellular matrix has been historically difficult to diagnose due to lack of specific clinical manifestations and necessity of biopsy to demonstrate amyloid deposition. However,advances in cardiovascular imaging techniques have facilitated earlier recognition of this disease. In addition,while once thought of as incurable,treatment strategies are emerging for cardiac amyloidosis,making early diagnosis essential.CASE SUMMARY We outline the case of a 73 years old African American female who was admitted with sudden onset shortness of breath and found to be in cardiogenic shock.Cardiac amyloidosis was suspected due to discordance between electrocardiogram and echocardiogram findings and this was subsequently confirmed with the aid of scintigraphy and an endomyocardial biopsy.CONCLUSION Our objective is to highlight the diagnostic evaluation and clinical implications of cardiac amyloidosis.展开更多
1 Introduction Cardiac amyloid(CA)is characterized by inexorably progressive heart failure making early diagnosis and treatment imperative.This article will review the pathophysiology,clinical presentation,diagnosis,p...1 Introduction Cardiac amyloid(CA)is characterized by inexorably progressive heart failure making early diagnosis and treatment imperative.This article will review the pathophysiology,clinical presentation,diagnosis,prognostication and treatment of patients with CA.2 Pathophysiology CA is the result of extracellular deposition of a misfolded protein into cardiac tissue,forming insoluble aggregations of rigid,nonbranching 10 nm wide fibrils.This causes impaired cardiac function by disrupting cardiac architecture,direct myotoxicity and ischemic injury secondary to infiltration of intramyocardial vessels.展开更多
This study described the radiological features on echocardiography and MRI specific to cardiac amyloidosis confirmed on biopsy. Eleven cases of biopsy-proven cardiac amyloidosis were retrospectively reviewed in this s...This study described the radiological features on echocardiography and MRI specific to cardiac amyloidosis confirmed on biopsy. Eleven cases of biopsy-proven cardiac amyloidosis were retrospectively reviewed in this study. All patients underwent biopsy, cardiac MRI and echocardiography. The main echocardiography and MRI findings were as follows: diffuse ventricular and septum wall thickening, atrial enlargement, pericardial effusion, restricted left ventricular (LV) systolic and diastolic function, characteristic granular sparkling of myocardium. MRI revealed a characteristic pattern of global subendocardial late enhancement, extending in varying degrees into the neighboring myocardium. The findings agreed with the infiltration distribution of amyloid protein. Typical abnormalities seen on echocardiography and MRI should have important diagnostic and prognostic value of cardiac amyloidosis. MRI should be considered in the diagnosis of cardiac amyloidosis if echocardiographic features are suspicious.展开更多
Amyloidosis, an infiltrative disease characterized by deposition of high molecular weight insoluble protein has a low detection rate and poor prognosis with congestive heart failure being the most common cause of deat...Amyloidosis, an infiltrative disease characterized by deposition of high molecular weight insoluble protein has a low detection rate and poor prognosis with congestive heart failure being the most common cause of death. Although definitive diagnosis is based on endomyocardial biopsy, this is rarely performed due to procedural risk and sampling error. Traditional non-invasive imaging, such as transthoracic echocardiography (TTE) and electrocardiogram (ECG) lacks sensitivity detecting the disease in its early stages. This case report demonstrates the utility of cardiac Magnetic Resonance Imaging in addition to traditional imaging modalities in this often clinically challenging problem.展开更多
Cardiac infiltration of amyloid fibril results in progressive cardiomyopathy with a grave prognosis and results in cardiac diseases such as congestive heart disease, cardiomyopathy, valvular heart disease, and arrhyth...Cardiac infiltration of amyloid fibril results in progressive cardiomyopathy with a grave prognosis and results in cardiac diseases such as congestive heart disease, cardiomyopathy, valvular heart disease, and arrhythmias. We present a rare case of cardiac amyloidosis initially misdiagnosed as syndrome X in which recurrent chest pain and progressive heart failure could be managed finally by heart transplantation.展开更多
Introduction: Cardiac amyloidosis is one of the several systemic manifestations of amyloidosis. It is a relatively uncommon disease. Patients with cardiac amyloidosis clinically present with rapidly progressive heart ...Introduction: Cardiac amyloidosis is one of the several systemic manifestations of amyloidosis. It is a relatively uncommon disease. Patients with cardiac amyloidosis clinically present with rapidly progressive heart failure. Case Presentation: We present a case of 64-year-old man with multiple episodes of chest discomfort accompanied sometimes with dyspnoea for the past several months. A non-specific ST-T changes with low voltage limb leads on ECG, a diastolic dysfunction with preserved ejection fraction on echocardiography, increased troponin and BNP, increased serum λ/κ light chain ratio led us to highly suspect amyloidosis with cardiac involvement. Abdominal fat pad biopsy confirmed the amyloid type change of subcutaneous fat, with immunohistochemistry revealing Lambda positive. After confirmed diagnosis of primary systemic amyloidosis with cardiac involvement, patient completed six cycles of chemotherapy with bortezomib and dexamethasone. The patient showed significant improvements after chemotherapy and supportive therapy. Conclusion: Amyloidosis is relatively fatal disease. Patients often have a short life expectancy after symptom onset. Early diagnosis is crucial to proper management and better prognosis.展开更多
Background: Amyloidosis is a disease characterized by the deposition of fibrillar proteins in tissues. The nature of the protein defines the type of amyloidosis. Cardiac involvement is most often secondary to deposits...Background: Amyloidosis is a disease characterized by the deposition of fibrillar proteins in tissues. The nature of the protein defines the type of amyloidosis. Cardiac involvement is most often secondary to deposits of transthyretin and immunoglobulin light chains. Treatment depends on the type of amyloidosis. Cardiac light chain amyloidosis is a medical emergency. Aim: To highlight the importance of an early diagnosis of cardiac light chain amyloidosis. Case Presentation: We report the case of an 88-year-old hypertensive female patient with sustained atrial fibrillation and recurrent heart failure, in whom echocardiography showed concentric left ventricle hypertrophy with mildly reduced left ventricle ejection fraction (LVEF) to 45%. Bone scintigraphy was normal. Serum analysis showed increased lambda free light chains. Accessory salivary gland biopsy revealed weak Kappa light chain staining and clear overexpression of lambda light chain deposits. The diagnosis of stage 3B cardiac amyloidosis secondary to lambda light chain myeloma was made. After a multidisciplinary meeting, it was decided to start treatment with DARATUMUMAB + LENALIDOMIDE. Patient’s general condition deteriorated with the occurrence of febrile pancytopenia. Chemotherapy was stopped and management was limited to comfort care until the patient’s death. Conclusion: Cardiac light-chain amyloidosis must be diagnosed early as it can be rapidly fatal.展开更多
Amyloidosis is an infiltrative disease caused by extracellular protein deposition that has accumulated a lot of scientific production in recent years.Different types of amyloidosis can affect the heart.Transthyretin a...Amyloidosis is an infiltrative disease caused by extracellular protein deposition that has accumulated a lot of scientific production in recent years.Different types of amyloidosis can affect the heart.Transthyretin amyloidosis and light chain amyloidosis are the two most common types of cardiac amyloidosis.These entities have a poor prognosis,so accurate diagnostic techniques are imperative for determining an early therapeutic approach.Recent advances in cardiac imaging and diagnostic strategies show that these tools are safe and can avoid the use of invasive diagnostic techniques to histological confirmation,such as endomyocardial biopsy.We performed a review on the diagnostic and prognostic implications of different cardiac imaging techniques in cardiac amyloidosis.We mainly focus on reviewing echocardiography,cardiac magnetic resonance,computed tomography and nuclear imaging techniques and the different safety measurements that can be done with each of them.展开更多
Objective To explore the potential role of native cardiac T1 mapping by cardiac magnetic resonance for detection of cardiac amyloidosis（CA）.Methods Twenty patients with cardiac amyloidosis（with definite cardiac inv...Objective To explore the potential role of native cardiac T1 mapping by cardiac magnetic resonance for detection of cardiac amyloidosis（CA）.Methods Twenty patients with cardiac amyloidosis（with definite cardiac involvement based on standard biomarkers and echo cardiographic criteria）were studied at our hospital展开更多
文摘Cardiac amyloidosis is a progressive disease characterized by the buildup of amyloid fibrils in the extracellular space of the heart.It is divided in 2 main types,immunoglobulin light chain amyloidosis and transthyretin amyloidosis(ATTR),and ATTR amyloidosis is further divided in 2 subtypes,non-hereditary wild type ATTR and hereditary mutant variant amyloidosis.Incidence and prevalence of ATTR cardiac amyloidosis is increasing over the last years due to the improvements in diagnostic methods.Survival rates are improving due to the development of novel therapeutic strategies.Tafamidis is the only disease-modifying approved therapy in ATTR amyloidosis so far.However,the most recent advances in medical therapies have added more options with the potential to become part of the therapeutic armamentarium of the disease.Agents including acoramidis,eplontersen,vutrisiran,patisiran and anti-monoclonal antibody NI006 are being investigated on cardiac function in large,multicenter controlled trials which are expected to be completed within the next 2-3 years,providing promising results in patients with ATTR cardiac amyloidosis.However,further and ongoing research is required in order to improve diagnostic methods that could provide an early diagnosis,as well as survival and quality of life of these patients.
文摘BACKGROUND Pulmonary hypertension is a rare cardiopulmonary disease,with an insidious onset that usually worsens rapidly.Amyloid light chain(AL)amyloidosis is a rare systemic disease caused by extracellular deposition of pathologic,insoluble,and proteinaceous fibrils in organs and tissues;however,it is difficult to diagnose given its varied and nonspecific symptoms.To date,rare cases of amyloidosis with pulmonary hypertension have been reported.Of note,the optimal treatments for cardiac amyloidosis complicated with pulmonary hypertension remain unclear.CASE SUMMARY We report a case of a 51-year-old woman who presented with progressively worsening dyspnea.Transthoracic echocardiography indicated severe pulmonary hypertension.Twenty-seven months after first admission,the patient returned with symptoms of progressive heart failure.A myocardial tissue sample stained with Congo red was positive,and the patient was ultimately diagnosed with AL amyloidosis with cardiac involvement.CONCLUSION Although pulmonary hypertension may be idiopathic,it is frequently associated with other conditions.In rare cases,pulmonary hypertension can be a complication of AL amyloidosis,which should be seriously considered in any adult presenting with nonspecific signs or symptoms of cardiac distress.
文摘Purpose: Transthyretin cardiac amyloidosis (ATTR-CA) has been linked to many extra-cardiac manifestations including bilateral carpal tunnel syndrome (CTS). The aim of this study is to analyze patients with bilateral CTS to identify patients with high-risk features or “red flags” for ATTR-CA, identify if systematic screening was done for ATTR-CA and define opportunities for improved detection. Methods: Out of >5000 patients with bilateral CTS evaluated in a single tertiary care center in Southeast Michigan (2010-2016), we retrospectively studied a focused population of patients: men > 50 years and women > 60 years old with bilateral CTS and atrial fibrillation (n = 295). Baseline demographic, comorbidities, and electrocardiographic and echocardiographic findings were analyzed. A high-risk group suspicious for ATTR-CA was identified as patients with bilateral CTS, atrial fibrillation, and concomitant “red flags” including heart failure and left ventricular hypertrophy. Results: Out of 295 patients, 51.2% were female, 75.6% were White, and 22.4% were African American. Upon comparing the high-risk group (n = 67) with the remaining study population (n = 228), both diagnosis of ATTR-CA and mortality were higher among the high-risk group (7.5% vs 0.4% and 43.3% vs 24.6%, respectively, P = 0.003). Conclusions: A substantial number of bilateral CTS patients had additional “red flags” warranting formal evaluation for ATTR-CA;however, systematic evaluation for cardiac amyloidosis was not performed in many patients. This emphasizes that Multidisciplinary collaboration is needed to create a systematic workflow and to raise awareness amongst cardiologists and other physicians for suspecting ATTR-CA in bilateral CTS patients who have additional “red flags”.
文摘Cardiac amyloidosis is a heterogeneous and challenging diagnostic disease with poor prognosis that is now being altered by introduction of new therapies.Echocardiography remains the first-line imaging tool, and when disease is suspected on echocardiography, cardiac magnetic resonance imaging and nuclear imaging play critical roles in the non-invasive diagnosis and evaluation of cardiac amyloidosis. Advances in multi-modality cardiac imaging allowing earlier diagnosis and initiation of novel therapies have significantly improved the outcomes in these patients. Cardiac imaging also plays important roles in the risk stratification of patients presenting with cardiac amyloidosis. In the current review, we provide a clinical and imaging focused update, and importantly outline the imaging protocols, diagnostic and prognostic utility of multimodality cardiac imaging in the assessment of cardiac amyloidosis.
文摘Introduction: Cardiac amyloidosis is a rare and under-diagnosed disease. The objective of this study was to collect cases of cardiac amyloidosis in patients hospitalized in the cardiology department of the Centre Hospitalier Intercommunal Alençon-Mamers (CHICAM). Patients and Methods: This was a retrospective descriptive study of the records of patients diagnosed with cardiac amyloidosis. The study took place in the cardiology department of the CHICAM over 12 months (from January 1, 2020 to December 31, 2020). We included the files of patients in whom the diagnosis of cardiac amyloidosis was confirmed on the basis of clinical, echocardiographic, biological, scintigraphic and cardiac MRI evidence. Results: Seven patients were included. The mean age was 86.71 years. Six men for one woman. Most of the patients were in heart failure. There was one case of periorbital ecchymosis. Troponinemia was increased in three patients, NTproBNP was always high with a mean of 1000 ng/L. Cardiac echography showed a hyperbright septum in four cases, concentric hypertrophy of the left ventricle with a mean interventricular septum in diastole of 13.85 mm. Six cases were senile transthyretin amyloidosis (TTR), one case was mutated transthyretin amyloidosis (TTRm). Two cases of death were noted. Conclusion: Cardiac amyloidosis has a poor prognosis due to delayed diagnosis.
文摘BACKGROUND Cardiac amyloidosis,a disease caused by the precipitation of amyloid proteins in the myocardial extracellular matrix has been historically difficult to diagnose due to lack of specific clinical manifestations and necessity of biopsy to demonstrate amyloid deposition. However,advances in cardiovascular imaging techniques have facilitated earlier recognition of this disease. In addition,while once thought of as incurable,treatment strategies are emerging for cardiac amyloidosis,making early diagnosis essential.CASE SUMMARY We outline the case of a 73 years old African American female who was admitted with sudden onset shortness of breath and found to be in cardiogenic shock.Cardiac amyloidosis was suspected due to discordance between electrocardiogram and echocardiogram findings and this was subsequently confirmed with the aid of scintigraphy and an endomyocardial biopsy.CONCLUSION Our objective is to highlight the diagnostic evaluation and clinical implications of cardiac amyloidosis.
文摘1 Introduction Cardiac amyloid(CA)is characterized by inexorably progressive heart failure making early diagnosis and treatment imperative.This article will review the pathophysiology,clinical presentation,diagnosis,prognostication and treatment of patients with CA.2 Pathophysiology CA is the result of extracellular deposition of a misfolded protein into cardiac tissue,forming insoluble aggregations of rigid,nonbranching 10 nm wide fibrils.This causes impaired cardiac function by disrupting cardiac architecture,direct myotoxicity and ischemic injury secondary to infiltration of intramyocardial vessels.
文摘This study described the radiological features on echocardiography and MRI specific to cardiac amyloidosis confirmed on biopsy. Eleven cases of biopsy-proven cardiac amyloidosis were retrospectively reviewed in this study. All patients underwent biopsy, cardiac MRI and echocardiography. The main echocardiography and MRI findings were as follows: diffuse ventricular and septum wall thickening, atrial enlargement, pericardial effusion, restricted left ventricular (LV) systolic and diastolic function, characteristic granular sparkling of myocardium. MRI revealed a characteristic pattern of global subendocardial late enhancement, extending in varying degrees into the neighboring myocardium. The findings agreed with the infiltration distribution of amyloid protein. Typical abnormalities seen on echocardiography and MRI should have important diagnostic and prognostic value of cardiac amyloidosis. MRI should be considered in the diagnosis of cardiac amyloidosis if echocardiographic features are suspicious.
文摘Amyloidosis, an infiltrative disease characterized by deposition of high molecular weight insoluble protein has a low detection rate and poor prognosis with congestive heart failure being the most common cause of death. Although definitive diagnosis is based on endomyocardial biopsy, this is rarely performed due to procedural risk and sampling error. Traditional non-invasive imaging, such as transthoracic echocardiography (TTE) and electrocardiogram (ECG) lacks sensitivity detecting the disease in its early stages. This case report demonstrates the utility of cardiac Magnetic Resonance Imaging in addition to traditional imaging modalities in this often clinically challenging problem.
文摘Cardiac infiltration of amyloid fibril results in progressive cardiomyopathy with a grave prognosis and results in cardiac diseases such as congestive heart disease, cardiomyopathy, valvular heart disease, and arrhythmias. We present a rare case of cardiac amyloidosis initially misdiagnosed as syndrome X in which recurrent chest pain and progressive heart failure could be managed finally by heart transplantation.
文摘Introduction: Cardiac amyloidosis is one of the several systemic manifestations of amyloidosis. It is a relatively uncommon disease. Patients with cardiac amyloidosis clinically present with rapidly progressive heart failure. Case Presentation: We present a case of 64-year-old man with multiple episodes of chest discomfort accompanied sometimes with dyspnoea for the past several months. A non-specific ST-T changes with low voltage limb leads on ECG, a diastolic dysfunction with preserved ejection fraction on echocardiography, increased troponin and BNP, increased serum λ/κ light chain ratio led us to highly suspect amyloidosis with cardiac involvement. Abdominal fat pad biopsy confirmed the amyloid type change of subcutaneous fat, with immunohistochemistry revealing Lambda positive. After confirmed diagnosis of primary systemic amyloidosis with cardiac involvement, patient completed six cycles of chemotherapy with bortezomib and dexamethasone. The patient showed significant improvements after chemotherapy and supportive therapy. Conclusion: Amyloidosis is relatively fatal disease. Patients often have a short life expectancy after symptom onset. Early diagnosis is crucial to proper management and better prognosis.
文摘Background: Amyloidosis is a disease characterized by the deposition of fibrillar proteins in tissues. The nature of the protein defines the type of amyloidosis. Cardiac involvement is most often secondary to deposits of transthyretin and immunoglobulin light chains. Treatment depends on the type of amyloidosis. Cardiac light chain amyloidosis is a medical emergency. Aim: To highlight the importance of an early diagnosis of cardiac light chain amyloidosis. Case Presentation: We report the case of an 88-year-old hypertensive female patient with sustained atrial fibrillation and recurrent heart failure, in whom echocardiography showed concentric left ventricle hypertrophy with mildly reduced left ventricle ejection fraction (LVEF) to 45%. Bone scintigraphy was normal. Serum analysis showed increased lambda free light chains. Accessory salivary gland biopsy revealed weak Kappa light chain staining and clear overexpression of lambda light chain deposits. The diagnosis of stage 3B cardiac amyloidosis secondary to lambda light chain myeloma was made. After a multidisciplinary meeting, it was decided to start treatment with DARATUMUMAB + LENALIDOMIDE. Patient’s general condition deteriorated with the occurrence of febrile pancytopenia. Chemotherapy was stopped and management was limited to comfort care until the patient’s death. Conclusion: Cardiac light-chain amyloidosis must be diagnosed early as it can be rapidly fatal.
文摘Amyloidosis is an infiltrative disease caused by extracellular protein deposition that has accumulated a lot of scientific production in recent years.Different types of amyloidosis can affect the heart.Transthyretin amyloidosis and light chain amyloidosis are the two most common types of cardiac amyloidosis.These entities have a poor prognosis,so accurate diagnostic techniques are imperative for determining an early therapeutic approach.Recent advances in cardiac imaging and diagnostic strategies show that these tools are safe and can avoid the use of invasive diagnostic techniques to histological confirmation,such as endomyocardial biopsy.We performed a review on the diagnostic and prognostic implications of different cardiac imaging techniques in cardiac amyloidosis.We mainly focus on reviewing echocardiography,cardiac magnetic resonance,computed tomography and nuclear imaging techniques and the different safety measurements that can be done with each of them.
文摘Objective To explore the potential role of native cardiac T1 mapping by cardiac magnetic resonance for detection of cardiac amyloidosis（CA）.Methods Twenty patients with cardiac amyloidosis（with definite cardiac involvement based on standard biomarkers and echo cardiographic criteria）were studied at our hospital
