Current Challenges of Cardiac Amyloidosis Awareness among Romanian Cardiologists

Abstract

Cardiac amyloidosis (CA) is a restrictive cardiomyopathy characterized by deposition of amyloid in the myocardium and recent studies revealed it is more frequently seen than we thought. Advances in diagnosis and treatment have been made over the last few years that make it desirable to diagnose CA without delay, and that may require extra education. An online survey was conducted among cardiologists from Romania, representing the first assessment of the knowledge of CA among them, with 195 cardiologists answering the questionnaire. There was a wide variation in their knowledge regarding CA. Our participants had limited experience with CA and reported a significant delay between first cardiac symptoms and diagnosis. We address the gaps in knowledge that were identified as educational opportunities in the main identified areas: prevalence and treatment of wild type transthyretin amyloidosis (ATTRwt), prevalence of variant transthyretin amyloidosis (ATTRv) in Romania, diagnosis of CA, the delay in CA diagnosis and available treatment options. Awareness among cardiologists is the most important challenge in diagnosing CA. Romanian cardiologists are partially aware of this topic, but there are still gaps in their knowledge. Educational programs can improve screening of patients with a high suspicion for this progressive condition the prognosis of which has been dramatically changed by the new treatment options.

Awareness of Nuclear Medicine Physicians in Romania Regarding the Diagnostic of Cardiac Amyloidosis—A Survey-Based Study

“…Another study related the knowledge to the diagnosis and therapy of CA among Romanian cardiologists was also conducted by Adam et al The study’s findings highlight that the Romanian cardiologists are partially aware of CA, but several misconceptions about the diagnostic suspicion of CA and therapy, emphasize the need to update the knowledge related to this pathological condition [ 22 ].…”

World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM)

“…Positron emission tomography (PET) scans with amyloid-specific tracers, such as 11 C-Pittsburgh compound B (PiB) [142], 18 F-florbetaben [143], and 18 F-florbetapir [144], detect cardiac amyloidosis. Nevertheless the uptake occurs not only in ATTR amyloidosis tissues but also in AL amyloidosis [142][143][144].…”

“…Management via specific treatments are now available and others are underway [3,11,12]. However, the condition remains misdiagnosed [13,14] and underdiagnosed [5] since awareness of the disease among clinicians and also in the community is far from desirable [15][16][17][18][19][20].…”

Role of Diphosphonates Bone Scintigraphy in Correlation with Biomarkers for a Personalized Approach to ATTR Cardiac Amyloidosis in North-Eastern Romania

“…According to the European Society of Cardiology's (ESC) 2021 guidelines, the gold standard method for detecting cardiac amyloidosis is endomyocardial biopsy. However, due to the disadvantages of this method (its inability to diagnose the subtype involved, its low accessibility and its highly invasive nature), alternative diagnostic algorithms have been tested [3][4][5]. When cardiac amyloidosis is suspected (more than 12 mm thickening of the left ventricle wall in a patient over 65 years of age, in addition to at least one "red flag" criterion), the guidelines recommend simultaneous bone scan examination and free light chain dosage (FLC) [6].…”

“…When cardiac amyloidosis is suspected (more than 12 mm thickening of the left ventricle wall in a patient over 65 years of age, in addition to at least one "red flag" criterion), the guidelines recommend simultaneous bone scan examination and free light chain dosage (FLC) [6]. The so-called "red flags" guide the physicians' diagnostic algorithm towards a possible cardiac amyloidosis: patients over 65 years of age that may suffer from heart failure, aortic stenosis, normotensive or hypotensive but with a history of hypertension, autonomous dysfunction and sensory-motor impairment, peripheral polyneuropathy, proteinuria, ecchymosis, bilateral carpal tunnel syndrome, bicep muscle tendon rupture, transmural or subendocardic LGE or increased extracellular volume, reduced longitudinal strain with apical sparing, microvoltage, pseudoinfarct pattern, atrioventricular conduction disorders and family history [3,5,7].…”