Myocardial Fibrosis Identified by Cardiac Magnetic Resonance Late Gadolinium Enhancement Is Associated With Adverse Ventricular Mechanics and Ventricular Tachycardia Late After Fontan Operation

• Published in: Journal of the American College of Cardiology 2010-04, Vol.55 (16), p.1721-1728
• Main Authors: Rathod, Rahul H., MD, Prakash, Ashwin, MD, Powell, Andrew J., MD, Geva, Tal, MD
• Format: Article
• Language: English
• Subjects: Acquisitions & mergers; Age; Cardiac arrhythmia; Cardiology; Cardiovascular; Cardiovascular disease; congenital heart disease; Contrast Media - administration & dosage; Electrocardiography; Endomyocardial Fibrosis - complications; Endomyocardial Fibrosis - diagnosis; Endomyocardial Fibrosis - physiopathology; Female; Follow-Up Studies; Fontan procedure; Fontan Procedure - adverse effects; Gadolinium DTPA - administration & dosage; Heart; Heart attacks; Heart Defects, Congenital - surgery; Heart Ventricles - pathology; Heart Ventricles - physiopathology; Humans; Injections, Intravenous; Internal Medicine; magnetic resonance imaging; Magnetic Resonance Imaging, Cine - methods; Male; Multivariate analysis; Myocardial Contraction - physiology; myocardial delayed enhancement; myocardial fibrosis; Myocardium - pathology; Prognosis; Pulmonary arteries; Retrospective Studies; Stroke Volume - physiology; Studies; Surgery; Tachycardia, Ventricular - diagnosis; Tachycardia, Ventricular - etiology; Tachycardia, Ventricular - physiopathology; Time Factors; Veins & arteries; Ventricular Dysfunction - diagnosis; Ventricular Dysfunction - etiology; Ventricular Dysfunction - physiopathology; Young Adult
• ISSN: 0735-1097; 1558-3597
• DOI: 10.1016/j.jacc.2009.12.036

Objectives The purpose of this study was to evaluate the relationship between myocardial fibrosis identified by cardiac magnetic resonance (CMR) and ventricular performance and arrhythmias in patients late after the Fontan operation. Background Patients who have undergone the Fontan palliation may develop ventricular dysfunction and arrhythmias, but the mechanisms and risk factors are poorly defined. Methods All patients who have had a Fontan operation and a CMR study with the myocardial delayed-enhancement technique from January 2002 to November 2008 were retrospectively identified. Results Of 90 patients (mean age at study was 23.1 ± 10.9 years), 25 (28%) had positive late gadolinium enhancement (LGE) in the ventricular myocardium. Patients with positive LGE had lower mean ejection fraction (45% vs. 56%; p < 0.001), increased median end-diastolic volume (100 ml/body surface area [BSA]1.3 vs. 82 ml/BSA1.3 ; p = 0.004), increased median ventricular mass i (63 g/BSA1.3 vs. 45 g/BSA1.3 ; p < 0.001), higher frequency of regional wall motion abnormalities (52% vs. 28%; p = 0.05), and higher frequency of nonsustained ventricular tachycardia (NSVT) (36% vs. 11%; p = 0.01). Multivariate regression analysis demonstrated that more extensive positive LGE, expressed as percent LGE of total myocardial mass, was associated with lower ejection fraction (p = 0.002), increased end-diastolic volume (p < 0.001), increased mass i (p < 0.001), and a higher frequency of NSVT (odds ratio 1.2; 95% confidence interval: 1.1 to 1.4; p = 0.006). Conclusions In this cohort of late Fontan survivors, myocardial fibrosis was common and associated with adverse ventricular mechanics and a higher prevalence of NSVT. Further studies are warranted to examine the utility of LGE for risk stratification and treatment of ventricular arrhythmia and dysfunction in Fontan patients.