Researchers of this large retrospective study evaluated how conventional heart failure medications are used in patients with transthyretin cardiac amyloidosis (ATTR-CA) and whether they are associated with survival. Researchers analyzed 2,371 patients diagnosed at the UK National Amyloidosis Centre between 2000 and 2022, excluding those with ATTR-polyneuropathy because autonomic neuropathy and postural hypotension can affect medication tolerance. At diagnosis, 55.4% of patients were taking beta-blockers, 57.4% were taking angiotensin-converting enzyme inhibitors (ACEis) or angiotensin II receptor blockers (ARBs), and 39.0% were taking mineralocorticoid receptor antagonists (MRAs). Patients receiving these medications generally had more comorbidities and more advanced cardiac disease, including worse New York Heart Association class, higher N-terminal pro-B-type natriuretic peptide, lower estimated glomerular filtration rate, reduced functional capacity, and worse echocardiographic measures.

The researchers found that beta-blockers and ACEi/ARBs were usually prescribed at low doses and were frequently discontinued, likely because ATTR-CA physiology can make patients sensitive to lower heart rate, vasodilation, and hypotension. In contrast, MRAs were rarely discontinued. In multivariable and propensity-matched analyses, MRA use was independently associated with lower mortality in the overall ATTR-CA population and especially in patients with left ventricular ejection fraction (LVEF) >40%. Low-dose beta-blockers were associated with lower mortality in patients with LVEF ≤40%, while ACEi/ARBs showed no clear survival benefit. The authors conclude that MRAs and low-dose beta-blockers may have a role in selected ATTR-CA patients, but these findings are hypothesis-generating and need confirmation in prospective randomized trials.

Reference: Ioannou A, Massa P, Patel RK, et al. Conventional heart failure therapy in cardiac ATTR amyloidosis. Eur Heart J. 2023 Aug 14;44(31):2893-2907. doi: 10.1093/eurheartj/ehad347. Erratum in: Eur Heart J. 2024 Apr 7;45(14):1251. doi: 10.1093/eurheartj/ehae140.

Authors of this review explain that delayed diagnosis remains one of the biggest barriers to timely transthyretin amyloid cardiomyopathy (ATTR-CM) treatment because symptoms are often nonspecific and commonly mistaken for hypertensive cardiomyopathy, hypertrophic cardiomyopathy, ischemic heart disease, aortic stenosis, or heart failure with preserved ejection fraction (HFpEF). The authors stress that ATTR-CM should stay high on the differential when patients have left ventricular wall thickening plus “red flags,” such as age over 60, family history of neuropathy or early heart failure, HFpEF without hypertension, low-flow aortic stenosis, low voltage or conduction abnormalities on electrocardiogram, or intolerance to standard heart-failure medications.

The article reviews the expanding ATTR-CM treatment landscape, including TTR stabilizers, TTR silencers, gene-editing approaches, and emerging amyloid-clearing therapies. Tafamidis, acoramidis, and vutrisiran now have clinical data supporting benefits such as improved survival, fewer cardiovascular hospitalizations, and preservation of functional capacity. Patisiran and eplontersen remain especially relevant to ATTR polyneuropathy and ongoing cardiomyopathy research. The authors also highlight investigational strategies such as CRISPR-Cas9 gene editing and monoclonal antibodies designed to clear misfolded TTR deposits. Overall, the review frames ATTR-CM care as shifting from symptom management alone to earlier diagnosis, disease modification, and potentially amyloid removal or durable TTR suppression.

Reference: Wanniarachchige D, Khan S, Pan S. Transthyretin Amyloid Cardiomyopathy Treatment: An Updated Review. J Clin Med. 2025 Aug 28;14(17):6089. doi: 10.3390/jcm14176089.

This study evaluated how well apical sparing pattern (ASP) on echocardiographic longitudinal strain predicts cardiac amyloidosis (CA) in a broader real-world population. Researchers retrospectively reviewed 466 adults who had transthoracic echocardiography plus additional testing, such as cardiac MRI, PYP scan, or endomyocardial biopsy, within an 18-month period. ASP was identified in 33 patients. Only 9 had confirmed CA and 2 were considered highly probable, giving ASP a positive predictive value of 33% for confirmed or highly probable CA. Most patients with ASP—64%—had no evidence of CA after further evaluation.

The findings suggest that ASP is a useful screening clue, but not a stand-alone diagnostic marker. Patients with ASP and confirmed CA tended to be older and had greater posterior wall thickness and relative wall thickness, while the degree of apical sparing itself did not distinguish those with CA from those without it. The authors note that ASP can occur in other conditions, including aortic stenosis and rare infiltrative or metabolic processes, so clinicians should interpret it in context. Still, because one-third of patients with ASP had confirmed or highly probable CA, the authors conclude that further testing is often justified, especially in older patients or those with increased left ventricular wall thickness.

Reference: Wali E, Gruca M, Singulane C, et al. How Often Does Apical Sparing of Longitudinal Strain Indicate the Presence of Cardiac Amyloidosis? Am J Cardiol. 2023 Sep 1;202:12-16. doi: 10.1016/j.amjcard.2023.06.022.