Scholarlink

<p>Cardiac amyloidosis is an infiltrative cardiomyopathy caused by extracellular deposition of amyloid fibrils, most commonly from immunoglobulin light chain (AL) or transthyretin (ATTR) proteins. Cardiac involvement dictates prognosis, with restrictive physiology, diastolic dysfunction, and conduction disease leading to progressive heart failure. Management of heart failure in cardiac amyloidosis differs fundamentally from conventional heart failure strategies. Due to preload dependence, autonomic dysfunction, and poor tolerance of standard therapies, treatment focuses on cautious volume control using low-dose diuretics, while β-blockers and ACE inhibitors, are used sparingly. Mineralocorticoid receptor antagonists are generally well tolerated and commonly used, with monitoring for hyperkalemia and renal dysfunction. Disease-specific therapy has revolutionized outcomes: daratumumab-based chemotherapy in AL amyloidosis achieves rapid and deep hematologic responses, improving cardiac biomarkers, imaging parameters, and survival. In the ATTR-subtype, transthyretin stabilizers and silencers significantly reduce mortality, cardiovascular hospitalizations, and functional decline, especially when initiated early. Atrial fibrillation and conduction disease are common; anticoagulation is indicated irrespective of CHA₂DS₂-VASc score. Ιmplantable cardioverter-defibrillator and cardiac resynchronization therapy are reserved for standard indications, with limited survival benefit. Inotropes serve as palliative or bridge therapy in end-stage disease, while transplantation remains viable in selected patients with controlled systemic disease. Emerging TTR stabilizers, gene silencers, and fibril-clearing antibodies may further transform prognosis. Advances in imaging, digital monitoring, and multidisciplinary care aim to enhance early detection and individualized management. Timely diagnosis, evidence-based therapy, and coordinated multidisciplinary care are essential to optimize survival and quality of life in cardiac amyloidosis.</p> Graphical Abstract <p></p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Tailoring heart failure management in cardiac amyloidosis

  • 期刊论文

  • 发表日期：

    2026年4月10日

查看全文
  • Ernesto Ruiz Duque,
  • Nikolaos-lason Tepetes,
  • Chris Kourek,
  • Ibrahim Mortada,
  • Adamantia Papamichail,
  • Efstathios Kastritis,
  • Kimon Stamatelopoulos,
  • Alexandros Briasoulis,
  • Rabea Asleh

摘要

Cardiac amyloidosis is an infiltrative cardiomyopathy caused by extracellular deposition of amyloid fibrils, most commonly from immunoglobulin light chain (AL) or transthyretin (ATTR) proteins. Cardiac involvement dictates prognosis, with restrictive physiology, diastolic dysfunction, and conduction disease leading to progressive heart failure. Management of heart failure in cardiac amyloidosis differs fundamentally from conventional heart failure strategies. Due to preload dependence, autonomic dysfunction, and poor tolerance of standard therapies, treatment focuses on cautious volume control using low-dose diuretics, while β-blockers and ACE inhibitors, are used sparingly. Mineralocorticoid receptor antagonists are generally well tolerated and commonly used, with monitoring for hyperkalemia and renal dysfunction. Disease-specific therapy has revolutionized outcomes: daratumumab-based chemotherapy in AL amyloidosis achieves rapid and deep hematologic responses, improving cardiac biomarkers, imaging parameters, and survival. In the ATTR-subtype, transthyretin stabilizers and silencers significantly reduce mortality, cardiovascular hospitalizations, and functional decline, especially when initiated early. Atrial fibrillation and conduction disease are common; anticoagulation is indicated irrespective of CHA₂DS₂-VASc score. Ιmplantable cardioverter-defibrillator and cardiac resynchronization therapy are reserved for standard indications, with limited survival benefit. Inotropes serve as palliative or bridge therapy in end-stage disease, while transplantation remains viable in selected patients with controlled systemic disease. Emerging TTR stabilizers, gene silencers, and fibril-clearing antibodies may further transform prognosis. Advances in imaging, digital monitoring, and multidisciplinary care aim to enhance early detection and individualized management. Timely diagnosis, evidence-based therapy, and coordinated multidisciplinary care are essential to optimize survival and quality of life in cardiac amyloidosis.

Graphical Abstract