Transthyretin amyloid cardiomyopathy in severe aortic stenosis submitted to valve replacement: a multicenter study
Creators: name: Pereira, Tamara
name: Fernandes, Raquel Menezes
name: Mata, Emídio
name: Azevedo, Olga
name: Bento, Dina
name: Jesus, Ilídio
name: Lourenço, António
Publisher: name: Taylor & Francis
Year: 2024
Subjects: subject: Space Science
subject: Medicine
subject: Cell Biology
subject: Immunology
subject: FOS: Clinical medicine
subject: Cancer

description: <b>Aim:</b> To evaluate the prevalence of TTR amyloid cardiomyopathy (ATTR-CM) in severe aortic stenosis (SAS) patients, and to determine the independent predictors of major adverse events (MAE). <b>Patients &amp; methods:</b> 91 SAS patients &gt;65 years with an interventricular septum thickness ≥12.5 mm were referred for aortic valve replacement (AVR). 99mTc-DPD scintigraphy was applied to diagnose ATTR-CM, in the absence of monoclonal protein. <b>Results:</b> ATTR-CM was found in 11%. 78% of patients underwent AVR, but only 2 had ATTR-CM. There were no significant differences in the composite of all cause-mortality or cardiovascular hospitalizations. Lower left ventricle ejection fraction and not performing AVR were independent predictors of MAE. <b>Conclusion:</b> Not performing AVR was an independent predictor of MAE, regardless the ATTR-CM diagnosis. Our study aimed to evaluate the number of people with severe narrowing of the aortic valve (SAS) and damage to the heart muscle caused to the deposition of filamentous structures composed of TTR (ATTR-CM), and to determine the independent predictors of severe undesirable medical occurrences (MAE). 91 patients &gt;65 years with SAS and increased thickness of the heart muscle were referred to perform an aortic valve prosthesis implantation (AVR). A nuclear medicine exam was used to diagnose ATTR-CM, after excluding the deposition of filamentous structures composed of blood proteins in the heart muscle. ATTR-CM was found in 11%. 78% of patients underwent AVR, but only two had ATTR-CM. There were no significant differences in both death rate from all causes or hospitalizations from cardiovascular causes. A lower percentage of blood pumped out of the heart in each beat and not performing AVR independently predicted the occurrence of MAE in SAS patients, regardless the ATTR-CM diagnosis. The prevalence of TTR amyloid cardiomyopathy (ATTR-CM) in our population of severe degenerative aortic stenosis (AS) patients was 11%. AS patients with concomitant ATTR-CM were older, had a higher frequency of carpal tunnel syndrome, a higher median NT-proBNP, and a lower voltage on electrocardiogram (ECG). They had a more severe left ventricular hypertrophy (LVH), more right ventricular hypertrophy (RVH) and low-flow low-gradient AS, worse LV diastolic dysfunction, higher pulmonary artery systolic pressure, lower left ventricular global longitudinal strain (LV-GLS) and a higher frequency of the relative apical sparing pattern. An increased awareness of ATTR-CM red-flags is needed to promote its timely diagnosis. Patients with ATTR-CM were less submitted to aortic valve replacement (AVR) than patients with lone AS, mainly due to higher frequency of refusals to intervention in the AS + ATTR-CM group, despite similar surgical risk. We did not find a statistically significant association of ATTR-CM with the composite of all cause-mortality or cardiovascular hospitalizations in our population, despite a relative higher frequency of adverse events in ATTR-CM patients. Not performing AVR was an independent predictor of all-cause mortality and cardiovascular hospitalizations in AS patients, regardless of the presence of ATTR-CM. AVR should not be withheld in patients with both AS and ATTR-CM. There is an urgent need to improve the healthcare response of transcatheter AVR to AS patients.