Background: even if prevalent among patients with severe aortic stenosis (aS), the clinical suspicion for transthyretin cardiac amyloidosis (ATTR-CA) remains difficult in this subset. We report our single center experience on ATTR-CA detection among TaVr candidates to provide insights on the prevalence and clinical features of dual pathology as compared to lone aS. MeTHodS: Consecutive severe aS patients undergoing transcatheter aortic valve replacement (TaVr) evaluation at a single center were prospectively included. Those with suspected aTTr-Ca based on clinical assessment underwent 99m Tc-3,3diphosphono-1,2-propanodicarboxylic acid (dPd) bone scintigraphy. The raiSe score, a novel screening tool with high sensitivity for aTTr-Ca in aS, was retrospectively calculated to rule-out aTTr-Ca in the remaining patients. Patients were categorized as follow: “ATTR-CA+”: patients with confirmed ATTR-CA at DPD bone scintigraphy; “ATTR-CA-”: patients with negative DPD bone scintigraphy or a negative RAISE score; “ATTR-CA indeterminate”: patients not undergoing ATTRCa assessment with a positive raiSe score. The characteristics of aTTr-Ca+ and aTTr-Ca- patients were compared. RESULTS: Of 107 included patients, ATTR-CA suspicion was posed in 13 patients and confirmed in six. Patients were categorized as follow: 6 (5.6%) aTTr-Ca+, 79 (73.8%) aTTr-Ca-, 22 (20.6%) aTTr-Ca indeterminate. excluding aTTr-Ca indeterminate patients, the prevalence of aTTr-Ca was 7.1% (95% Ci 2.6-14.7%). as compared to aTTr-Ca – patients, aTTr-Ca + patients were older, had higher procedural risk and more extensive myocardial and renal damage. They had higher left ventricle mass index and lower eCg voltages, translating into a lower voltage to mass ratio. Moreover, we describe for the first time bifascicular block as an ECG feature highly specific of patients with dual pathology (50.0% vs. 2.7%, P<0.001). of note, pericardial effusion was rarely found in patients with lone aS (16.7% vs. 1.2%, P=0.027). No difference in procedural outcomes was observed between groups. CoNClUSioNS: among severe aS patients, aTTr-Ca is prevalent and presents with phenotypic features that may aid to differentiate it from lone aS. a clinical approach based on routine search of amyloidosis features might lead to selective dPd bone scintigraphy with a satisfactory positive predictive value.
Transthyretin cardiac amyloidosis in patients with severe aortic stenosis undergoing transcatheter aortic valve replacement: experience of a single center
Gallone G.;Bongiovanni C.;Bruno F.;Landra F.;Scudeler L.;de Filippo O.;Califaretti E.;Cioffi M.;Pidello S.;Vairo A.;Frea S.;Giorgi M.;Alunni G.;SALIZZONi S.;Conrotto F.;D'ASCENZo F.;Rinaldi M.;de Ferrari G. M.
2024-01-01
Abstract
Background: even if prevalent among patients with severe aortic stenosis (aS), the clinical suspicion for transthyretin cardiac amyloidosis (ATTR-CA) remains difficult in this subset. We report our single center experience on ATTR-CA detection among TaVr candidates to provide insights on the prevalence and clinical features of dual pathology as compared to lone aS. MeTHodS: Consecutive severe aS patients undergoing transcatheter aortic valve replacement (TaVr) evaluation at a single center were prospectively included. Those with suspected aTTr-Ca based on clinical assessment underwent 99m Tc-3,3diphosphono-1,2-propanodicarboxylic acid (dPd) bone scintigraphy. The raiSe score, a novel screening tool with high sensitivity for aTTr-Ca in aS, was retrospectively calculated to rule-out aTTr-Ca in the remaining patients. Patients were categorized as follow: “ATTR-CA+”: patients with confirmed ATTR-CA at DPD bone scintigraphy; “ATTR-CA-”: patients with negative DPD bone scintigraphy or a negative RAISE score; “ATTR-CA indeterminate”: patients not undergoing ATTRCa assessment with a positive raiSe score. The characteristics of aTTr-Ca+ and aTTr-Ca- patients were compared. RESULTS: Of 107 included patients, ATTR-CA suspicion was posed in 13 patients and confirmed in six. Patients were categorized as follow: 6 (5.6%) aTTr-Ca+, 79 (73.8%) aTTr-Ca-, 22 (20.6%) aTTr-Ca indeterminate. excluding aTTr-Ca indeterminate patients, the prevalence of aTTr-Ca was 7.1% (95% Ci 2.6-14.7%). as compared to aTTr-Ca – patients, aTTr-Ca + patients were older, had higher procedural risk and more extensive myocardial and renal damage. They had higher left ventricle mass index and lower eCg voltages, translating into a lower voltage to mass ratio. Moreover, we describe for the first time bifascicular block as an ECG feature highly specific of patients with dual pathology (50.0% vs. 2.7%, P<0.001). of note, pericardial effusion was rarely found in patients with lone aS (16.7% vs. 1.2%, P=0.027). No difference in procedural outcomes was observed between groups. CoNClUSioNS: among severe aS patients, aTTr-Ca is prevalent and presents with phenotypic features that may aid to differentiate it from lone aS. a clinical approach based on routine search of amyloidosis features might lead to selective dPd bone scintigraphy with a satisfactory positive predictive value.
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