A Review of Cardiac Amyloidosis: Presentation, Diagnosis, and Treatment

Pour-Ghaz, Issa; Bath, Anandbir; Kayali, Sharif; Alkhatib, Deya; Yedlapati, Neeraja; Rhea, Isaac; Khouzam, Rami N.; Jefferies, John L.; Nayyar, Mannu

doi:10.1016/j.cpcardiol.2022.101366

Cited by 13 publications

(33 citation statements)

References 102 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…On the other hand, medications that reduce the heart rate, and in particular beta-blockers, are poorly tolerated due to repression of the compensatory increase in heart rate and resultant further reduction of the cardiac output. Likewise, calcium antagonists are considered harmful in cardiac amyloidosis patients [ 25 , 26 , 27 ]. Nevertheless, at low doses, they may be considered for their antihypertensive action during early disease.…”

Section: Supraventricular Tachyarrhythmiasmentioning

confidence: 99%

Arrhythmic Manifestations of Cardiac Amyloidosis: Challenges in Risk Stratification and Clinical Management

Laptseva

Rossi

Sudano

et al. 2023

JCM

View full text Add to dashboard Cite

Amylodiosis is a systemic disease characterized by extracellular deposits of insoluble amyloid in various tissues and organs. Cardiac amyloidosis is a frequent feature of the disease, causing a progressive, restrictive type of cardiomyopathy, and is associated with adverse clinical outcomes and increased mortality. The typical clinical presentation in patients with cardiac amyloidosis is heart failure (HF) with preserved ejection fraction. Most patients present with typical symptoms and signs of HF, such as exertional dyspnea, pretibial edema, pleural effusions and angina pectoris due to microcirculatory dysfunction. However, patients may also frequently encounter various arrhythmias, such as atrioventricular nodal block, atrial fibrillation and ventricular tachyarrhythmias. The management of arrhythmias in cardiac amyloidosis patients with drugs and devices is often a clinical challenge. Moreover, predictors of life-threatening arrhythmic events are not well defined. This review intends to give a deepened insight into the arrhythmic features of cardiac amyloidosis by discussing the pathogenesis of these arrhythmias, addressing the challenges in risk stratification and strategies for management in these patients.

show abstract

Section: Supraventricular Tachyarrhythmiasmentioning

confidence: 99%

Arrhythmic Manifestations of Cardiac Amyloidosis: Challenges in Risk Stratification and Clinical Management

Laptseva

Rossi

Sudano

et al. 2023

JCM

View full text Add to dashboard Cite

show abstract

“…Với sự phát triển của các phương tiện hình ảnh học như siêu âm tim, cộng hưởng từ tim (CMR -Cardiac magnetic resonance), xạ hình và các chỉ dấu sinh học, CA có thể được chẩn đoán sớm. Mặc dù vậy vẫn còn đó những điểm chưa thống nhất giữa các tổ chức tim mạch lớn trên thế giới về các chiến lược chẩn đoán CA [32], [33].…”

Section: đặT Vấn đềunclassified

“…Thuật ngữ amyloidosis (từ tiếng Hy Lạp ἄμυλον: amylon, "bột") đã được biến đến từ thế kỷ 19 bởi Rudolf Virchow, ông là một nhà nghiên cứu bệnh học người Đức [6]. CA thường dùng để mô tả một nhóm bệnh lý di truyền trong gia đình hoặc mắc phải đặc trưng bởi sự lắng đọng ngoại bào của các sợi protein không tan [6], [32].…”

Section: Tổng Quan 21 Giới Thiệuunclassified

Tổng quan bệnh tim thoái hóa dạng bột trong thực hành lâm sàng

Nguyễn Minh,

Phạm Duy,

Nguyễn Tuấn

2022

PNTJMP

View full text Add to dashboard Cite

Amyloidosis là một bệnh lý ảnh hưởng đến nhiều cơ quan do sự lắng đọng bất thường của các protein trong các mô khác nhau. Biểu hiện lâm sàng phụ thuộc vào thể bệnh và vị trí lắng đọng của các sợi amyloid. Các triệu chứng hầu hết không đặc hiệu, bao gồm mệt mỏi, khó thở, sụt cân, phù ngoại vi, xu hướng dễ chảy máu và các đặc điểm khác như bệnh thần kinh tự chủ hoặc bệnh thần kinh ngoại biên. Biểu hiện tim mạch của amyloidosis cũng rất đa dạng như rối loạn nhịp, hạ huyết áp tư thế và thậm chí là suy tim. Chẩn đoán bệnh cơ tim thoái hóa dạng bột thường khó khăn do biểu hiện lâm sàng không đặc hiệu, diễn tiến bệnh âm thầm kéo dài và tương đối hiếm gặp. Vì vậy bệnh có thể không được chẩn đoán trong nhiều năm dẫn đến gia tăng tỉ lệ tử vong. Đánh giá bệnh cơ tim thoái hóa dạng bột đòi hỏi phải tiếp cận đa mô thức bằng các phương tiện hình ảnh học như siêu âm tim, cộng hưởng từ tim, hình ảnh học hạt nhân và các chỉ dấu sinh học. Tuy nhiên, tiêu chuẩn vàng để chẩn đoán amyloidosis vẫn là sinh thiết. Cho đến nay các chiến lược chẩn đoán bệnh cơ tim thoái hóa dạng bột vẫn chưa được thống nhất giữa các tổ chức tim mạch lớn trên thế giới. Abstract Amyloidosis is a disease that affects many organs due to abnormal protein deposition in various tissues. Clinical manifestations depend on the type of disease and the location of amyloid infiltration. Symptoms are usually nonspecific and include fatigue, dyspnea, weight loss, peripheral edema, bleeding tendency, and other autonomic or peripheral neuropathy features. Also, the cardiovascular manifestations of amyloidosis are highly variable, including cardiac arrhythmias, orthostatic hypotension, and even heart failure. Diagnosis of cardiac amyloidosis can be difficult due to nonspecific presentation, persistent silencing, and relative rarity. Amyloidosis goes undiagnosed for years, increasing mortality. Evaluation of cardiac amyloidosis requires a multimodal approach using echocardiography, cardiac magnetic resonance, nuclear imaging, and many biomarkers. However, biopsy remains the gold standard for diagnosing amyloidosis. To date, the diagnosis of cardiac amyloidosis remains divided among major cardiovascular organizations.

show abstract

“…The fibrils in ATTR amyloidosis consist of misfolded TTR forming due to dissociation of either the wild-type protein (ATTRwt) or facilitated by mutations in the TTR gene (ATTRv) ( 1 – 4 , 6 – 8 ). Both forms of CA may be difficult to diagnose due to non-specific symptoms and overlap with other cardiomyopathies causing delayed initiation of treatment and, consequently, poorer prognosis ( 9 , 10 ). Survival was significantly better among patients with AL amyloidosis diagnosed in < 6 months from symptom onset with >52% of patients surviving over the 5-year period of the study than for those whose diagnosis took longer who also had significantly increased risk of death as shown by >63% of patients dying during the study period ( 9 ).…”

Section: Introductionmentioning

confidence: 99%

“…Survival was significantly better among patients with AL amyloidosis diagnosed in < 6 months from symptom onset with >52% of patients surviving over the 5-year period of the study than for those whose diagnosis took longer who also had significantly increased risk of death as shown by >63% of patients dying during the study period ( 9 ). Undiagnosed and delayed diagnosis of CA results in high morbidity and high mortality whereas early diagnosis has both clinical and quality of life (QoL) benefits for patients with AL, ATTRwt, or ATTRv CA ( 10 – 12 ). Using a disease simulation model, early diagnosis and timely treatment have been shown to extend the calculated life expectancy from the onset of symptoms by more than 5 and more than 7 years among patients with ATTRwt and ATTRv amyloidosis, respectively ( 12 ).…”

Section: Introductionmentioning

confidence: 99%

Changing paradigm in the treatment of amyloidosis: From disease-modifying drugs to anti-fibril therapy

Quarta

Fontana

Damy

et al. 2022

Front. Cardiovasc. Med.

View full text Add to dashboard Cite

Cardiac amyloidosis is a rare, debilitating, and usually fatal disease increasingly recognized in clinical practice despite patients presenting with non-specific symptoms of cardiomyopathy. The current standard of care (SoC) focuses on preventing further amyloid formation and deposition, either with anti-plasma cell dyscrasia (anti-PCD) therapies in light-chain (AL) amyloidosis or stabilizers of transthyretin (TTR) in transthyretin amyloidosis (ATTR). The SoC is supplemented by therapies to treat the complications arising from organ dysfunction; for example, heart failure, arrhythmia, and proteinuria. Advancements in treatments have improved patient survival, especially for those whose disease is detected and for whom treatment is initiated at an early stage. However, there still are many unmet medical needs, particularly for patients with severe disease for whom morbidity and mortality remain high. There currently are no approved treatments to reverse amyloid infiltration and deplete the amyloid fibrils already deposited in organs, which can continue to cause progressive dysfunction. Anti-fibril therapies aimed at removing the deposited fibrils are being investigated for safety and efficacy in improving outcomes for patients with severe disease. However, there is no clinical evidence yet that removing deposited amyloid fibrils will improve organ function, thereby improving quality of life or extending life. Nevertheless, anti-fibril therapies are actively being investigated in clinical trials to evaluate their ability to complement and synergize with current SoC.

show abstract

A Review of Cardiac Amyloidosis: Presentation, Diagnosis, and Treatment

Cited by 13 publications

References 102 publications

Arrhythmic Manifestations of Cardiac Amyloidosis: Challenges in Risk Stratification and Clinical Management

Arrhythmic Manifestations of Cardiac Amyloidosis: Challenges in Risk Stratification and Clinical Management

Tổng quan bệnh tim thoái hóa dạng bột trong thực hành lâm sàng

Changing paradigm in the treatment of amyloidosis: From disease-modifying drugs to anti-fibril therapy

Contact Info

Product

Resources

About