Author + information
- Received April 29, 2015
- Revision received September 7, 2015
- Accepted September 10, 2015
- Published online January 1, 2016.
- Chun-An Chen, MD, PhD,
- Susan M. Dusenbery, MD, MS,
- Anne Marie Valente, MD,
- Andrew J. Powell, MD and
- Tal Geva, MD∗ ()
- Department of Cardiology, Boston Children’s Hospital, and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts
- ↵∗Reprint requests and correspondence:
Dr. Tal Geva, Department of Cardiology, Boston Children’s Hospital, 300 Longwood Avenue, Boston, Massachusetts 02115.
Objectives The aim of this study was to investigate the extent of diffuse myocardial fibrosis by measuring left (LV) and right ventricular (RV) extracellular volume fraction (ECV) in patients with repaired tetralogy of Fallot (rTOF) and to explore its association with ventricular remodeling, hemodynamic load, and clinical parameters.
Background Focal myocardial fibrosis is prevalent in patients with rTOF. However, little is known about the extent of diffuse myocardial fibrosis and its clinical implications in this population.
Methods We measured ECV by pre- and post-gadolinium T1 measurements using a 1.5-T scanner in 84 patients with rTOF (median age 23.3 years). LV ECV was determined by averaging values from 6 short-axis mid-ventricular segments, and RV ECV was calculated by averaging values from the anterior-inferior and the diaphragmatic RV wall segments.
Results LV ECV above the upper limit of normal (>28%) was observed in 11 patients and for RV ECV (>41%) in 9 patients. LV ECV correlated positively with RV ECV (r = 0.54; p < 0.001). Greater RV ECV was associated with female gender, lower RV mass-to-volume ratio, lower RV outflow tract pressure gradient, and having volume overload as the predominant hemodynamic burden (all p < 0.001). Similar associations were observed with LV ECV. In multivariable analysis, increased LV ECV was independently associated with arrhythmia, adjusting for age and RV mass index (odds ratio: 5.69; p = 0.031).
Conclusions In this cohort, LV and RV ECV values were positively correlated, indicating an adverse ventricular-ventricular interaction at the tissue level. Increased ECV was associated with RV volume overload and arrhythmia. These findings may lead to future studies exploring the role of ECV in improving risk stratification and guiding therapeutic interventions.
- cardiac magnetic resonance imaging
- congenital heart disease
- diffuse fibrosis
- extracellular volume fraction
- tetralogy of Fallot
Dr. Chen was a visiting scholar from the Department of Cardiology, National Taiwan University Children’s Hospital, Taipei, Taiwan, and was supported by grant NSC 102-2314-B-002-060-MY3 from the Ministry of Science and Technology, Taiwan. The study was supported in part by the Higgins Family Noninvasive Cardiac Imaging Research Fund at Boston Children’s Hospital. Dr. Geva has served as a consultant for Medtronic. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Drs. Chen and Dusenbery contributed equally to this work.
- Received April 29, 2015.
- Revision received September 7, 2015.
- Accepted September 10, 2015.
- American College of Cardiology Foundation