: Heart Failure Management of Patients with Amyloid Cardiomyopathy
CA has a restrictive physiology, characterized by elevated filling pressures and low stroke volumes. Initially, patients present with preserved ejection fraction, which may decrease in late stages of disease. The use of diuretics is crucial in the treatment of patients with CA. The evidence of GDMT in the treatment of patients with CA is not very robust and is based on retrospective cohort analysis. Heart transplantation may be considered for patients with advanced HF. ACEI: angiotensin-converting enzyme inhibitor; ARB: angiotensin II receptor blocker; HFpEF: heart failure with preserved ejection fraction; HFrEF: heart failure with reduced ejection fraction; SGLT2i: sodium-glucose transport protein 2 inhibitors; CA: cardiac amyloidosis.
Abstract
Cardiac amyloidosis (CA) can lead to progressive heart failure (HF) by depositing insoluble amyloid fibrils within the myocardial extracellular space, resulting in an infiltrative and restrictive cardiomyopathy. Although CA was previously perceived as rare and incurable, recent advances in diagnostics and emerging therapies have been changing this outlook. It is crucial to spread awareness about CA to facilitate earlier diagnosis and proper therapeutic interventions, enhancing patient prognosis and survival. Currently, there is an estimated delay of 2 years from symptom onset to diagnosis, typically involving consultation with an average of 5 different professionals. Advances in cardiovascular imaging have facilitated earlier and more accurate diagnosis, reducing the necessity for invasive procedures, such as endomyocardial biopsy. Presently, tafamidis is the only drug that has been shown to offer prognostic benefits in ATTR-CA. Tafamidis is a highly specific medication targeting the circulating TTR protein, stabilizing the TTR tetramer to prevent its dissociation into amyloidogenic monomers that deposit in the myocardium. Alongside specific amyloidosis therapy, supportive HF treatment may be required; however, managing CA with medications typically used for HF with reduced ejection fraction (HFrEF) can be challenging due to potential intolerance. The effectiveness of guideline-directed medical therapy (GDMT) remains undetermined and still requires evaluation through randomized controlled clinical trials (RCCTs). Thus, the treatment cornerstone remains the judicious use of loop diuretics and mineralocorticoid receptor antagonists to control volume overload. Due to the safety profile, not adversely affecting hemodynamics or renal function, sodium-glucose transport protein 2 (SGLT2) inhibitors may be an effective treatment for CA, but they also still require evaluation through RCCTs.
Amyloidosis; Cardiomyopathies; Heart Failure; Left Ventricular Dysfunction
