Myocardial structural and functional changes in cardiac amyloidosis: insights from a prospective observational patient registry
Author:
Duca Franz1ORCID, Rettl René1ORCID, Kronberger Christina1, Binder Christina1, Mann Christopher1, Dusik Fabian1, Schrutka Lore1ORCID, Dalos Daniel1ORCID, Öztürk Begüm2, Dachs Theresa Marie1, Cherouny Bernhard1, Camuz Ligios Luciana1ORCID, Agis Hermine3, Kain Renate4, Koschutnik Matthias1ORCID, Donà Carolina1ORCID, Badr-Eslam Roza1, Kastner Johannes1, Beitzke Dietrich5ORCID, Loewe Christian5ORCID, Nitsche Christian1ORCID, Hengstenberg Christian1ORCID, Kammerlander Andreas Anselm1, Bonderman Diana12ORCID
Affiliation:
1. Department of Internal Medicine II, Division of Cardiology, Medical University of Vienna , Waehringer Guertel 18-20, 1090 Vienna , Austria 2. Division of Cardiology, Favoriten Clinic , Kundratstraße 3, 1100 Vienna , Austria 3. Department of Internal Medicine I, Division of Hematology, Medical University of Vienna , Vienna , Austria 4. Clinical Institute of Pathology, Medical University of Vienna , Vienna , Austria 5. Department of Bioimaging and Image-Guided Therapy, Division of Cardiovascular and Interventional Radiology, Medical University of Vienna , Vienna , Austria
Abstract
Abstract
Aims
The pathophysiological hallmark of cardiac amyloidosis (CA) is the deposition of amyloid within the myocardium. Consequently, extracellular volume (ECV) of affected patients increases. However, studies on ECV progression over time are lacking. We aimed to investigate the progression of ECV and its prognostic impact in CA patients.
Methods and results
Serial cardiac magnetic resonance (CMR) examinations, including ECV quantification, were performed in consecutive CA patients. Between 2012 and 2021, 103 CA patients underwent baseline and follow-up CMR, including ECV quantification. Median ECVs at baseline of the total (n = 103), transthyretin [(ATTR) n = 80], and [light chain (AL) n = 23] CA cohorts were 48.0%, 49.0%, and 42.6%, respectively. During a median period of 12 months, ECV increased significantly in all cohorts [change (Δ) +3.5% interquartile range (IQR): −1.9 to +6.9, P < 0.001; Δ +3.5%, IQR: −2.0 to +6.7, P < 0.001; and Δ +3.5%, IQR: −1.6 to +9.1, P = 0.026]. Separate analyses for treatment-naïve (n = 21) and treated (n = 59) ATTR patients revealed that the median change of ECV from baseline to follow-up was significantly higher among untreated patients (+5.7% vs. +2.3%, P = 0.004). Survival analyses demonstrated that median change of ECV was a predictor of outcome [total: hazard ratio (HR): 1.095, 95% confidence interval (CI): 1.047–1.0145, P < 0.001; ATTR: HR: 1.073, 95% CI: 1.015–1.134, P = 0.013; and AL: HR: 1.131, 95% CI: 1.041–1.228, P = 0.003].
Conclusion
The present study supports the use of serial ECV quantification in CA patients, as change of ECV was a predictor of outcome and could provide information in the evaluation of amyloid-specific treatments.
Publisher
Oxford University Press (OUP)
Subject
Cardiology and Cardiovascular Medicine,Radiology, Nuclear Medicine and imaging,General Medicine
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