Institute for Experimental Cardiovascular Medicine (Research Institute)
Department of Congenital Heart Defects and Pediatric Cardiology (Clinical Institute)
University Heart Center Freiburg – Bad Krozingen
The main aim of this project is the characterization of fibrotic remodeling in right ventricular myectomies of patients with congenital heart defects on an ultrastructural and electrophysiological level, and the association of these changes with non-invasive clinical parameters in order to improve patients’ risk stratification in the future.
This project is a collaboration between the Institute for Experimental Cardiovascular Medicine (IEKM) and the Department of Congenital Heart Defects and Pediatric Cardiology.
Wesley Jones, MSc student, 4D-imaging group at IEKM
Brigitte Stiller, Director of the Department of Congenital Heart Defects and Pediatric Cardiology
Johannes Kroll, Section head of Pediatric Cardiovascular Surgery
Congenital heart defects, such as tetralogy of Fallot and septal defects, are frequently associated with ventricular overload and/or systemic hypoxemia. Operative repair in infancy or childhood often allows for long-term survival and good quality of life. However, despite successful repair some patients remain at risk for severe arrhythmias, such as ventricular tachycardia and sudden cardiac death. Electrophysiological remodeling may stem from the pathology itself, as well as from the operative procedures. Especially in tetralogy of Fallot patients, recent MRI-based investigations and electroanatomical voltage mapping studies have associated ventricular arrhythmias with structural myocardial remodeling in the form of fibrosis. A better understanding of the cellular, ultrastructural, and electrophysiological basis and their interplay may enable earlier identification of those patients at risk, and allow for targeted and individualized preventive and therapeutic approaches.
Figure: Functional and structural investigations on right ventricular myecomies from tetralogy of Fallot patients. Intracellular action potential recordings performed on live tissue sample (A, B). Structural examinations reveal severe right ventricular fibrosis by conventional histology (C, red collagen, yellow myocardium) and second harmonic generation microscopy (D, depth-encoded label-free collagen visualization). Unpublished data by H. Fürniss.
SELECTED RECENT PUBLICATIONS
Manuscript in preparation:
E. M. Wülfers, J. Greiner, M. Giese, J. Madl, J. Kroll, B. Stiller, P. Kohl, E. A. Rog-Zielinska, and H. E. Fürniss. Quantitative Collagen Assessment in Right Ventricular Myectomies from Patients with Tetralogy of Fallot.
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