Clinical and Prognostic Implications of Right Ventricular Uptake on Bone Scintigraphy in Transthyretin Amyloid Cardiomyopathy

BACKGROUND: The extent of myocardial bone tracer uptake with technetium pyrophosphate, hydroxymethylene diphosphonate, and 3,3-diphosphono-1,2-propanodicarboxylate in transthyretin amyloid cardiomyopathy (ATTR-CM) might reflect cardiac amyloid burden and be associated with outcome.

METHODS: Consecutive patients with ATTR-CM who underwent diagnostic bone tracer scintigraphy with acquisition of whole-body planar and cardiac single-photon emission computed tomography (SPECT) images from the National Amyloidosis Centre and 4 Italian centers were included. Cardiac uptake was defined according to the Perugini classification: 0=absent cardiac uptake; 1=mild uptake less than bone; 2=moderate uptake equal to bone; and 3=high uptake greater than bone. Extent of right ventricular (RV) uptake was defined as focal (basal segment of the RV free wall only) or diffuse (extending beyond basal segment) on the basis of SPECT imaging. The primary outcome was all-cause mortality.

RESULTS: Among 1422 patients with ATTR-CM, RV uptake accompanying left ventricular uptake was identified by SPECT imaging in 100% of cases at diagnosis. Median follow-up in the whole cohort was 34 months (interquartile range, 21 to 50 months), and 494 patients died. By Kaplan-Meier analysis, diffuse RV uptake on SPECT imaging (n=936) was associated with higher all-cause mortality compared with focal (n=486) RV uptake (77.9% versus 22.1%; P<0.001), whereas Perugini grade was not associated with survival (P=0.27 in grade 2 versus grade 3). On multivariable analysis, after adjustment for age at diagnosis (hazard ratio [HR], 1.03 [95% CI, 1.02-1.04]; P<0.001), presence of the p.(V142I) TTR variant (HR, 1.42 [95% CI, 1.20-1.81]; P=0.004), National Amyloidosis Centre stage (each category, P<0.001), stroke volume index (HR, 0.99 [95% CI, 0.97-0.99]; P=0.043), E/e' (HR, 1.02 [95% CI, 1.007-1.03]; P=0.004), right atrial area index (HR, 1.05 [95% CI, 1.02-1.08]; P=0.001), and left ventricular global longitudinal strain (HR, 1.06 [95% CI, 1.03-1.09]; P<0.001), diffuse RV uptake on SPECT imaging (HR, 1.60 [95% CI, 1.26-2.04]; P<0.001) remained an independent predictor of all-cause mortality. The prognostic value of diffuse RV uptake was maintained across each National Amyloidosis Centre stage and in both wild-type and hereditary ATTR-CM (P<0.001 and P=0.02, respectively).

CONCLUSIONS: Diffuse RV uptake of bone tracer on SPECT imaging is associated with poor outcomes in patients with ATTR-CM and is an independent prognostic marker at diagnosis.

Errataetall:

CommentIn: Circulation. 2024 Apr 9;149(15):1169-1171. - PMID 38588337

Medienart:

E-Artikel

Erscheinungsjahr:

2024

Erschienen:

2024

Enthalten in:

Zur Gesamtaufnahme - volume:149

Enthalten in:

Circulation - 149(2024), 15 vom: 09. Apr., Seite 1157-1168

Sprache:

Englisch

Beteiligte Personen:

Porcari, Aldostefano [VerfasserIn]
Fontana, Marianna [VerfasserIn]
Canepa, Marco [VerfasserIn]
Biagini, Elena [VerfasserIn]
Cappelli, Francesco [VerfasserIn]
Gagliardi, Christian [VerfasserIn]
Longhi, Simone [VerfasserIn]
Pagura, Linda [VerfasserIn]
Tini, Giacomo [VerfasserIn]
Dore, Franca [VerfasserIn]
Bonfiglioli, Rachele [VerfasserIn]
Bauckneht, Matteo [VerfasserIn]
Miceli, Alberto [VerfasserIn]
Girardi, Francesca [VerfasserIn]
Martini, Anna Lisa [VerfasserIn]
Barbati, Giulia [VerfasserIn]
Costanzo, Egidio Natalino [VerfasserIn]
Caponetti, Angelo Giuseppe [VerfasserIn]
Paccagnella, Andrea [VerfasserIn]
Sguazzotti, Maurizio [VerfasserIn]
La Malfa, Giovanni [VerfasserIn]
Zampieri, Mattia [VerfasserIn]
Sciagrà, Roberto [VerfasserIn]
Perfetto, Federico [VerfasserIn]
Rowczenio, Dorota [VerfasserIn]
Gilbertson, Janet [VerfasserIn]
Hutt, David F [VerfasserIn]
Hawkins, Philip N [VerfasserIn]
Rapezzi, Claudio [VerfasserIn]
Merlo, Marco [VerfasserIn]
Sinagra, Gianfranco [VerfasserIn]
Gillmore, Julian D [VerfasserIn]

Links:

Volltext

Themen:

Amyloidosis
Journal Article
Prealbumin
Prognosis
Tomography, emission-computed, single-photon
Transthyretin

Anmerkungen:

Date Completed 10.04.2024

Date Revised 11.04.2024

published: Print-Electronic

CommentIn: Circulation. 2024 Apr 9;149(15):1169-1171. - PMID 38588337

Citation Status MEDLINE

doi:

10.1161/CIRCULATIONAHA.123.066524

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM36818174X