Myocardial fibrosis in Eisenmenger syndrome: a descriptive cohort study exploring associations of late gadolinium enhancement with clinical status and survival.

Craig S Broberg,Chad Carr,Michael A Gatzoulis,Konstantinos Dimopoulos,Sonya V Babu-Narayan,Sanjay K Prasad

doi:10.1186/1532-429x-16-32

Abstract

BackgroundA relationship between myocardial fibrosis and ventricular dysfunction has been demonstrated using late gadolinium enhancement (LGE) in the pressure-loaded right ventricle from congenital heart defects. In patients with Eisenmenger syndrome (ES), the presence of LGE has not been investigated. The aims of this study were to detect any myocardial fibrosis in ES and describe major clinical variables associated with the finding.MethodsFrom 45 subjects screened, 30 subjects (age 43 ± 13 years, 20 female) underwent prospective cardiovascular magnetic resonance with LGE to quantify biventricular volume and function as well as maximal and submaximal exercise during a single visit. Standard cine acquisitions were obtained for ventricular volume and function. Further imaging was performed after administration of 0.1 mmol/kg gadolinium contrast. Regions of LGE were evaluated qualitatively and quantitatively by manual contouring of identified areas, with total area expressed as a percentage of mass. Patients were followed prospectively (mean follow up 7.4 ± 0.4 years) and any deaths recorded. Patients with LGE findings were compared to those without.ResultsLGE was present in 22/30 (73%) patients, specifically in RV myocardium (70%), RV trabeculae (60%), LV myocardium (33%) or LV papillary muscles (30%), though in small amounts (mean 1.4% of total ventricular mass, range 0.16 – 6.0%). Those with any LGE were not different in age, history of arrhythmia, desaturation, nor hemoglobin, nor ventricular size, mass, or function. Exercise capacity was low, but also not different between those with and without LGE. Similarly no significant associations were found with amount of fibrosis. There were five deaths among patients with LGE, versus two in patients without, but no difference in survival (log rank =0.03, P = 0.85).ConclusionsMyocardial fibrosis by LGE is common in ES, though not extensive. The presence and quantity of LGE did not correlate with ventricular size, function, degree of cyanosis, exercise capacity, or survival in this pilot study. More data are clearly required before recommendations for routine use of LGE in these patients can be made.

Highlights

A relationship between myocardial fibrosis and ventricular dysfunction has been demonstrated using late gadolinium enhancement (LGE) in the pressure-loaded right ventricle from congenital heart defects
There was no difference in oxygen saturation, exercise capacity, ventricular size or function, or survival among those in whom LGE was or was not performed
In the present study of Eisenmenger patients, we found small amounts of fibrosis were very common, especially in the more vulnerable right ventricle (RV), indicating that mechanisms driving fibrosis are present

Summary

Introduction

A relationship between myocardial fibrosis and ventricular dysfunction has been demonstrated using late gadolinium enhancement (LGE) in the pressure-loaded right ventricle from congenital heart defects. LGE has been demonstrated in various congenital heart disease subgroups including those with tetralogy of Fallot, transposition of the great arteries, and following a Fontan palliation [3,4,5,6]. These studies find that LGE is associated with several clinical markers of cardiovascular dysfunction, including ventricular enlargement, systolic dysfunction, poorer NYHA class, arrhythmia, elevations of brain natriuretic peptide and lower exercise capacity. More diffuse microscopic fibrosis has been demonstrated in ES [8]

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