Work

Structural and Echocardiographic Abnormalities in Congenital Long QT Syndrome: A Review of the Literature

Year: 2026

Type: article

Abstract: Congenital Long QT Syndrome (LQTS) is a hereditary cardiac channelopathy defined by delayed ventricular repolarization and an elevated risk of life-threatening ventricular arrhythmias. Recent echocardiographic studies using speckle-tracking and strain imaging have identified subtle abnormalities in ventricular and atrial mechanics among LQTS patients, including reduced global longitudinal strain, impaired diastolic function, enlarged left atrial volumes and a consistently negative electromechanical window. These findings challenge the traditional concept of LQTS as solely an electrical disease and support evolving evidence of a subclinical cardiomyopathic phenotype. Left atrial remodeling, although less studied, may represent an underrecognized component of LQTS with potential implications for arrhythmia vulnerability and diastolic dysfunction. This review summarizes current evidence on electromechanical and structural cardiac involvement in congenital LQTS, highlights its diagnostic and clinical implications, and outlines future directions for research in this evolving field.

Source: Medicina

DOI: 10.3390/medicina62050829

Authors: Austė Markevičiūtė, Patricija Lapinskaitė, Mariola Kovalevska, Audronė Vaitiekienė, Diana Rinkūnienė

Institution: Lithuanian University of Health Sciences

Language: English

Topic: Cardiac electrophysiology and arrhythmias

Subfield: Cardiology and Cardiovascular Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $2,654