Usama Asghar, Samra Choudhry, Mahwish Jabeen, Khadija Mubashir, Ayrass Zia, Mehwish Sadique, Muhammad Jawad Wakil, Laiba Munawar
Shalamar Medical and Dental College. Faisalabad Medical University. Fatima Jinnah Medical University. Quaid e Azam Medical College. Al Aleem Medical College. Azra Naheed Medical College. Islam Medical and Dental College.
Pakistan
Cardiology in Review
Cardiol Rev 2026;
DOI: 10.1097/CRD.0000000000001450
Abstract
Myocardial fibrosis is increasingly recognized as a central determinant of adverse cardiac remodeling and long-term cardiovascular outcomes in children with congenital and acquired heart disease. Once considered an irreversible consequence of chronic myocardial injury, fibrosis is now understood to represent a dynamic biological process driven by persistent inflammation, neurohormonal activation, oxidative stress, and fibroblast-to-myofibroblast transformation. In pediatric populations, myocardial fibrosis develops across a wide spectrum of diseases including congenital heart disease, cardiomyopathies, myocarditis, pulmonary hypertension, and cancer therapy-related cardiotoxicity. Progressive accumulation of extracellular matrix disrupts myocardial architecture, impairs ventricular compliance, promotes electrical heterogeneity, and predisposes patients to ventricular dysfunction, arrhythmias, heart failure, and sudden cardiac death. Recent advances in cardiovascular magnetic resonance imaging have revolutionized fibrosis assessment. Late gadolinium enhancement identifies focal replacement fibrosis, whereas native T1 mapping and extracellular volume quantification enable sensitive detection of diffuse interstitial fibrosis before overt ventricular dysfunction develops. Complementary echocardiographic techniques, circulating biomarkers, and emerging artificial intelligence-based imaging analyses further enhance early risk stratification. Although no pediatric-specific antifibrotic therapy currently exists, growing evidence suggests that modulation of the renin-angiotensin-aldosterone system, neprilysin inhibition, sodium-glucose cotransporter-2 inhibitors, mineralocorticoid receptor antagonists, and novel anti-transforming growth factor-β strategies may attenuate myocardial remodeling in selected patients. This review summarizes contemporary mechanisms underlying pediatric myocardial fibrosis, highlights advances in imaging biomarkers, discusses clinical implications across major pediatric cardiovascular diseases, and explores emerging antifibrotic therapies that may redefine future management.
Category
Heart Dysfunction Associated with Pulmonary Vascular Disease (Right)
Review Articles Concerning Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No