ORIGINAL RESEARCH article
Front. Cardiovasc. Med.
Sec. Heart Failure and Transplantation
Volume 12 - 2025 | doi: 10.3389/fcvm.2025.1653950
This article is part of the Research TopicExploring Hypertrophic Cardiomyopathy and Cardiac AmyloidosisView all 4 articles
Prognostic implications of right ventricular to pulmonary artery uncoupling in cardiac amyloidosis
Provisionally accepted
Aiste Monika Jakstaite1
Anja Haenselmann1Samira Soltani1
Eleonora Angelini1
Michael Heuser2
Vega Gödecke3
Stefan Gingele4
Thomas Skripuletz4
Johann Bauersachs1Udo Bavendiek1
Dominik Berliner1*- 1Medizinische Hochschule Hannover Klinik fur Kardiologie und Angiologie, Hanover, Germany
- 2Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Hanover, Germany
- 3Medizinische Hochschule Hannover Klinik fur Nieren- und Hochdruckerkrankungen, Hanover, Germany
- 4Medizinische Hochschule Hannover Zentrum Neurologische Medizin, Hanover, Germany
Select one of your emails
You have multiple emails registered with Frontiers:
Notify me on publication
Please enter your email address:
If you already have an account, please login
You don't have a Frontiers account ? You can register here
Backround: Right ventricular-pulmonary arterial (RV-PA) uncoupling in cardiac amyloidosis (CA) has been underexplored, with focus mainly on TAPSE/PASP. This study aims to evaluate the association of various echocardiographic surrogates of RV–PA coupling with outcomes in cardiac transthyretin (ATTR-CA) and light-chain (AL-CA) amyloidosis. Methods: We analyzed RV–PA coupling in patients diagnosed with ATTR-CA and AL-CA at our center between 2014 and 2023. RV-PA coupling was assessed using TAPSE/PASP, FAC/PASP and RVFWS/PASP. The primary endpoint was all-cause mortality. Results: 120 patients (86% ATTR-CA, 14% AL-CA) were included in the study (median age 77 years, 88% male). During a median follow-up period of 23 (IQR: 15-34) months primary endpoint occurred in 25 patients (21%). The study population was stratified based on the ROC-derived TAPSE/PASP cutoff of <0.30 mm/mmHg demonstrating RV–PA uncoupling. Lower RV–PA coupling surrogates were independently associated with higher mortality (HR per +0.1 unit: TAPSE/PASP 0.74, 95% CI 0.59–0.93, p=0.011; FAC/PASP 0.87, 0.77–0.98, p=0.018; RVFWS/PASP 0.78, 0.63–0.97, p=0.024). TAPSE/PASP demonstrated the strongest prognostic discrimination (AUC 0.79, bootstrapped 95% CI 0.66–0.91), compared with FAC/PASP (AUC 0.75, 0.58–0.91) and RVFWS/PASP (AUC 0.72, 0.52–0.87). Conclusions: RV-PA uncoupling may be linked to a higher risk of all-cause mortality in CA. TAPSE/PASP outperformed numerically FAC/PASP and RVFWS/PASP in predicting long-term survival, although it did not clearly outperform established RV function parameters.
Keywords: right ventricular-pulmonary arterial coupling, Amyloidosis, transthyretin amyloidosis, Heart Failure, speckle-tracking echocardiography
Received: 25 Jun 2025; Accepted: 08 Sep 2025.
Copyright: © 2025 Jakstaite, Haenselmann, Soltani, Angelini, Heuser, Gödecke, Gingele, Skripuletz, Bauersachs, Bavendiek and Berliner. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Dominik Berliner, berliner.dominik@mh-hannover.de
Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.