A histopathologic schema to quantify the burden of cardiac amyloidosis: Relationship with survival and echocardiographic parameters.
cardiac amyloidosis
echocardiography
endomyocardial biopsy
longitudinal strain
Journal
Echocardiography (Mount Kisco, N.Y.)
ISSN: 1540-8175
Titre abrégé: Echocardiography
Pays: United States
ID NLM: 8511187
Informations de publication
Date de publication:
02 2019
02 2019
Historique:
received:
06
09
2018
revised:
16
11
2018
accepted:
04
12
2018
pubmed:
29
12
2018
medline:
6
6
2019
entrez:
29
12
2018
Statut:
ppublish
Résumé
Despite routine use of echocardiographic parameters to evaluate the severity of cardiac amyloidosis (CA), this methodology has not been well validated. We developed a histopathologic schema for quantifying CA burden and evaluated its relationship with clinical outcomes. Additionally, echocardiographic parameters were tested as potential noninvasive indices of CA burden. We retrospectively studied 59 patients with CA (17 light chain, 42 transthyretin) who underwent endomyocardial biopsies. Light microscopy with staining was used to categorize CA burden as mild-to-moderate (<50%) or high (≥50%). Kaplan-Meier survival analysis was performed for the two groups. In 34 patients with good-quality echocardiograms, we measured left ventricular volumes, ejection fraction (EF), interventricular septal thickness (IVSt), posterior wall thickness (PWt), LV mass, lateral e'-velocity, and global longitudinal strain (GLS). These parameters were compared between the two groups. Thirty-five patients had mild-to-moderate and 24 severe amyloid burden. Kaplan-Meier curves demonstrated a trend toward worse mortality with high CA burden, which was more common and associated with higher mortality specifically in transthyretin-type patients. Echocardiography-derived IVSt, PWt, and LV mass were directly related to CA burden, while LV EF, e'-velocity, and GLS magnitude were inversely related to CA burden. Our findings provided a signal that CA burden is a clinically important entity with potentially valuable prognostic information. Echocardiographic parameters of LV anatomy and function correlate with histopathologic burden of CA, which is inversely related to survival. Further studies are needed to determine whether these parameters could be used as imaging biomarkers of treatment-related changes in CA burden.
Sections du résumé
BACKGROUND
Despite routine use of echocardiographic parameters to evaluate the severity of cardiac amyloidosis (CA), this methodology has not been well validated. We developed a histopathologic schema for quantifying CA burden and evaluated its relationship with clinical outcomes. Additionally, echocardiographic parameters were tested as potential noninvasive indices of CA burden.
METHODS
We retrospectively studied 59 patients with CA (17 light chain, 42 transthyretin) who underwent endomyocardial biopsies. Light microscopy with staining was used to categorize CA burden as mild-to-moderate (<50%) or high (≥50%). Kaplan-Meier survival analysis was performed for the two groups. In 34 patients with good-quality echocardiograms, we measured left ventricular volumes, ejection fraction (EF), interventricular septal thickness (IVSt), posterior wall thickness (PWt), LV mass, lateral e'-velocity, and global longitudinal strain (GLS). These parameters were compared between the two groups.
RESULTS
Thirty-five patients had mild-to-moderate and 24 severe amyloid burden. Kaplan-Meier curves demonstrated a trend toward worse mortality with high CA burden, which was more common and associated with higher mortality specifically in transthyretin-type patients. Echocardiography-derived IVSt, PWt, and LV mass were directly related to CA burden, while LV EF, e'-velocity, and GLS magnitude were inversely related to CA burden.
CONCLUSIONS
Our findings provided a signal that CA burden is a clinically important entity with potentially valuable prognostic information. Echocardiographic parameters of LV anatomy and function correlate with histopathologic burden of CA, which is inversely related to survival. Further studies are needed to determine whether these parameters could be used as imaging biomarkers of treatment-related changes in CA burden.
Identifiants
pubmed: 30592782
doi: 10.1111/echo.14245
pmc: PMC8240650
mid: NIHMS1711975
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
285-291Subventions
Organisme : NHLBI NIH HHS
ID : T32 HL007381
Pays : United States
Informations de copyright
© 2018 Wiley Periodicals, Inc.
Références
Circulation. 2016 Jun 14;133(24):2404-12
pubmed: 27143678
Curr Hematol Malig Rep. 2018 Jun;13(3):212-219
pubmed: 29951831
Arch Intern Med. 1988 Mar;148(3):662-6
pubmed: 3341867
Heart. 1997 Jul;78(1):74-82
pubmed: 9290406
JACC Cardiovasc Imaging. 2016 Feb;9(2):126-38
pubmed: 26777222
Circulation. 2014 May 6;129(18):1840-9
pubmed: 24563469
Circulation. 2015 Oct 20;132(16):1570-9
pubmed: 26362631
N Engl J Med. 2018 Jul 05;379(1):22-31
pubmed: 29972757
JAMA Cardiol. 2016 Nov 1;1(8):880-889
pubmed: 27557400
N Engl J Med. 2018 Jul 5;379(1):11-21
pubmed: 29972753
J Am Soc Echocardiogr. 2015 Jan;28(1):1-39.e14
pubmed: 25559473
N Engl J Med. 2018 Sep 13;379(11):1007-1016
pubmed: 30145929
Curr Treat Options Cardiovasc Med. 2018 Apr 7;20(5):37
pubmed: 29627865
J Am Coll Cardiol. 2016 Jul 5;68(1):13-24
pubmed: 27364045