Curr Probl Cardiol. 2026 Apr 3:103338. doi: 10.1016/j.cpcardiol.2026.103338. Online ahead of print.
ABSTRACT
BACKGROUND: Transthyretin amyloidosis (ATTR) is a progressive multisystem disorder caused by extracellular deposition of amyloid fibrils derived from mutant (hATTR) or wild-type (ATTRwt) transthyretin. Cardiac involvement is the main determinant of prognosis and typically manifests as restrictive cardiomyopathy (ATTR-CM), leading to heart failure, arrhythmias, and conduction disturbances. However, ATTR frequently extends beyond the heart, involving peripheral and autonomic neuropathy, renal impairment, gastrointestinal dysfunction, musculoskeletal manifestations, anemia, nutritional decline, and frailty, all of which significantly contribute to clinical complexity, poorer outcomes, and reduced quality of life.
AIM: To highlight the clinical relevance of extracardiac organ involvement in ATTR-CM and to support a multidimensional, patient-centered approach to care.
EVIDENCE SYNTHESIS / MAIN FINDINGS: The effectiveness of disease-modifying therapies is maximized when incorporated into a comprehensive care model that balances therapeutic benefit with treatment tolerance and addresses both amyloid-related systemic organ damage and coexisting cardiac and extracardiac comorbidities. Optimal management therefore requires a coordinated multidisciplinary team including cardiology, neurology, nephrology, pulmonology, nutrition, geriatrics, psychology, rehabilitation, and social support services.
CONCLUSIONS: A comprehensive strategy integrating early diagnosis, systematic assessment of organ damage, frailty evaluation, and individualized therapeutic planning is essential to improve outcomes and deliver holistic, high-quality care for individuals with ATTR-CM. This review synthesizes current evidence on diagnosis and management of ATTR-CM and proposes a structured framework for evaluating and treating systemic complications associated with transthyretin amyloid disease.
PMID:41937005 | DOI:10.1016/j.cpcardiol.2026.103338