Cardiovascular complications in idiopathic pulmonary fibrosis and the emerging concept of the heart-lung fibrotic axis: a narrative review.
- 1Department of Pulmonology, Allergology and Pulmonary Oncology, Poznan University of Medical Sciences, Poznań, Poland.
- 2Department of Cardiology, Poznan University of Medical Sciences, Poznań, Poland.
Abstract
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease associated with high morbidity and mortality. Beyond respiratory failure, cardiovascular (CV) involvement is increasingly recognized as an integral component of IPF, contributing substantially to symptoms, functional decline, hospitalization, and survival. While pulmonary hypertension and right ventricular dysfunction remain the predominant CV manifestations, growing evidence suggests that subclinical myocardial dysfunction may precede overt CV disease. Collectively, these findings are consistent with a continuum of cardiopulmonary involvement, ranging from established CV complications to emerging subclinical myocardial abnormalities, rather than supporting a single unified fibrotic process. Shared profibrotic mechanisms, including transforming growth factor-β signaling, extracellular matrix remodeling, inflammation, oxidative stress, endothelial dysfunction, and neurohormonal activation, provide a biological rationale for the proposed heart-lung fibrotic axis, although its clinical relevance remains to be fully established. This review summarizes current evidence on clinical and subclinical CV manifestations in IPF, advances in multimodal diagnostic assessment, shared molecular mechanisms, and evolving therapeutic strategies. An integrated cardiopulmonary perspective may provide a framework for future studies aimed at improving risk stratification, earlier recognition of CV involvement, and development of therapeutic strategies.
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BibTeX
@article{graczyk2026cardiovascular,
title = {{Cardiovascular complications in idiopathic pulmonary fibrosis and the emerging concept of the heart-lung fibrotic axis: a narrative review.}},
author = {Patryk Graczyk and Daria Keller and Barbara Kuźnar-Kamińska},
journal = {Frontiers in medicine},
year = {2026},
month = oct,
doi = {10.3389/fmed.2026.1954683},
url = {https://europepmc.org/article/MED/42953011},
note = {CC BY},
}
RIS
TY - JOUR
TI - Cardiovascular complications in idiopathic pulmonary fibrosis and the emerging concept of the heart-lung fibrotic axis: a narrative review.
AU - Patryk Graczyk
AU - Daria Keller
AU - Barbara Kuźnar-Kamińska
PY - 2026
DA - 2026/10/14
JO - Frontiers in medicine
DO - 10.3389/fmed.2026.1954683
UR - https://europepmc.org/article/MED/42953011
AB - Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease associated with high morbidity and mortality. Beyond respiratory failure, cardiovascular (CV) involvement is increasingly recognized as an integral component of IPF, contributing substantially to symptoms, functional decline, hospitalization, and survival. While pulmonary hypertension and right ventricular dysfunction remain the predominant CV manifestations, growing evidence suggests that subclinical myocardial dysfunction may precede overt CV disease. Collectively, these findings are consistent with a continuum of cardiopulmonary involvement, ranging from established CV complications to emerging subclinical myocardial abnormalities, rather than supporting a single unified fibrotic process. Shared profibrotic mechanisms, including transforming growth factor-β signaling, extracellular matrix remodeling, inflammation, oxidative stress, endothelial dysfunction, and neurohormonal activation, provide a biological rationale for the proposed heart-lung fibrotic axis, although its clinical relevance remains to be fully established. This review summarizes current evidence on clinical and subclinical CV manifestations in IPF, advances in multimodal diagnostic assessment, shared molecular mechanisms, and evolving therapeutic strategies. An integrated cardiopulmonary perspective may provide a framework for future studies aimed at improving risk stratification, earlier recognition of CV involvement, and development of therapeutic strategies.
SN - 2296-858X
ER -