Project Description

Severe right ventricular fibrosis as revealed bz second harmonic generation microscopy (depth-encoded label-free collagen visualization). Unpublished data by H. Fürniss.

Hannah Fürniss, MD

Clinician Scientist
E: hannah.fuerniss@universitaets-herzzentrum.de

Institute for Experimental Cardiovascular Medicine (Research Institute)
Department of Congenital Heart Defects and Pediatric Cardiology (Clinical Institute)
University Heart Center Freiburg – Bad Krozingen
Mathildenstr. 1
79106 Freiburg
Germany

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.

TEAM

This project is a collaboration between the Institute for Experimental Cardiovascular Medicine (IEKM) and the Department of Congenital Heart Defects and Pediatric Cardiology.

  • Peter Kohl, Director of IEKM
  • Eva Rog-Zielinska, PI of 4D-Imaging Group at IEKM
  • Rémi Peyronnet, PI of Biophysics Group at IEKM
  • Hannah Fürniss, clinician scientist, 4D-Imaging Group at IEKM
  • Eike Wülfers, research scientist of Computational Modelling Group at IEKM
  • Joachim Greiner, research scientist of 4D-Imaging Group at IEKM
  • 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

PROJECT DESCRIPTION

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.

FUNDING