Abstract
Background: The prevalence of cardiac amyloidosis (CA) is unknown.Aims and Methods: We sought to (a) determine the prevalence of CA in unselected patients ≥75 years undergoing autopsy, (b) characterize cardiological profiles of CA and non-CA patients by providing clinical-histological correlations, and (c) compare their cardiological profiles. After dedicated staining, the localization (interstitial or vascular) and the distribution (non-diffuse or diffuse) of amyloid deposition were analyzed. Cardiological data at last evaluation were retrospectively assessed for the presence of CA red-flags.Results: CA (50% light chains, 50% transthyretin) was found in 43% (n = 24/56) of the autopsied hearts. Atria were involved in 96% of cases. Amyloid localized both at the perivascular and interstitial levels (95.5 and 85%, respectively) with a slightly predominant non-diffuse distribution (58% of cases). Compared to the other patients, CA patients had a more frequent history of heart failure (HF) (79 vs. 47%, p = 0.014), advanced NYHA functional class (III-IV 25 vs. 6%, p = 0.047), atrial fibrillation (68 vs. 36%, p = 0.019), discrepancy between QRS voltage and left ventricular (LV) thickness (70 vs. 12%, p < 0.001), thicker LV walls (15 vs. 11 mm, p < 0.001), enlarged left atrium (49 vs. 42 mm, p = 0.019) and restrictive filling pattern (56 vs. 19%, p = 0.020). The presence of right ventricular amyloidosis seemed to identify hearts with a higher amyloid burden. Among the CA patients, >30% had ≥3 echocardiographic red-flags of disease.Conclusion: CA can be found in 43% of autopsied hearts from patients ≥75 years old, especially in patients with HF, LV hypertrophy and atrial fibrillation.
Highlights
Cardiac amyloidosis (CA) is an increasingly recognized cause of heart failure (HF) and mortality, resulting from the progressive deposition of misfolded proteins [1, 2], mainly immunoglobulin light chains (AL) and transthyretin (ATTR) [3]
CA can be found in 43% of autopsied hearts from patients ≥75 years old, especially in patients with HF, left ventricular (LV) hypertrophy and atrial fibrillation
The main findings of our study are that (a) CA can be found in up to 43% (24/56) of hearts from unselected subjects ≥75 years old who underwent autopsies, (b) AL-CA was as frequent as ATTR-CA, (c) CA patients more frequently had a history of HF with advanced NYHA functional class, AF with more dilated atria and LV concentric hypertrophy with thicker walls, (d) patients with right ventricle (RV) amyloidosis had more severe cardiological profiles and more heavily infiltrated hearts at histology, (e) no patient received a diagnosis of CA, even though ECG-echo discrepancy and at least 3 red flags of CA were present in 70% and in more than 30% of patients with histologically proven CA, respectively
Summary
Cardiac amyloidosis (CA) is an increasingly recognized cause of heart failure (HF) and mortality, resulting from the progressive deposition of misfolded proteins [1, 2], mainly immunoglobulin light chains (AL) and transthyretin (ATTR) [3]. Most epidemiological data come from historical post-mortem studies that report a disarming prevalence of concealed CA in 25% of unselected adults >80 years old and in 32% of patients >75 years old with HF with preserved ejection fraction (HFpEF) [4]. These studies had a mainly histological focus; none of them were conducted on unselected populations, nor did they provide a correlation between histological and clinical data.
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