title: Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation.
abstract: OBJECTIVE
  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/BSA(1.3); p = 0.004), increased median ventricular mass(i) (63 g/BSA(1.3) vs. 45 g/BSA(1.3); 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.
authors.affiliation: Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
authors.forename: Rahul H
authors.initials: RH
authors.lastname: Rathod
authors.forename: Ashwin
authors.initials: A
authors.lastname: Prakash
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Powell
authors.forename: Tal
authors.initials: T
authors.lastname: Geva
mesh_terms: D003287:Contrast Media / Q000008:administration & dosage; D004562:Electrocardiography; D004719:Endomyocardial Fibrosis / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D019786:Gadolinium DTPA / Q000008:administration & dosage; D006330:Heart Defects, Congenital / Q000601:surgery; D006352:Heart Ventricles / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007275:Injections, Intravenous; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D009200:Myocardial Contraction / Q000502:physiology; D009206:Myocardium / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D013318:Stroke Volume / Q000502:physiology; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D013997:Time Factors; D018754:Ventricular Dysfunction / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D055815:Young Adult
chemical_list: D003287:Contrast Media; D019786:Gadolinium DTPA
references.citation: J Am Coll Cardiol. 2008 Jul 8;52(2):85-98
references.pmid: 18598886
references.citation: Lancet. 2003 Feb 1;361(9355):374-9
references.pmid: 12573373
references.citation: Circulation. 2005 Apr 26;111(16):2091-8
references.pmid: 15851616
references.citation: Circulation. 2009 Mar 17;119(10):1370-7
references.pmid: 19255342
references.citation: Circulation. 2008 Jan 1;117(1):85-92
references.pmid: 18071068
references.citation: Circulation. 2006 Jan 24;113(3):405-13
references.pmid: 16432072
references.citation: Am J Cardiol. 2005 Mar 1;95(5):688-91
references.pmid: 15721124
references.citation: J Am Coll Cardiol. 2005 Jan 4;45(1):98-103
references.pmid: 15629382
references.citation: Circulation. 2002 Mar 12;105(10):1189-94
references.pmid: 11889012
references.citation: J Magn Reson Imaging. 2003 Mar;17(3):323-9
references.pmid: 12594722
references.citation: J Am Coll Cardiol. 2004 Jun 16;43(12):2260-4
references.pmid: 15193690
references.citation: J Am Coll Cardiol. 2008 Jun 24;51(25):2414-21
references.pmid: 18565399
references.citation: J Appl Physiol (1985). 2005 Aug;99(2):445-57
references.pmid: 15557009
references.citation: J Am Coll Cardiol. 2008 Jul 8;52(2):99-107
references.pmid: 18598887
references.citation: Int J Cardiovasc Imaging. 2008 Jan;24(1):61-8
references.pmid: 17429755
references.citation: Circulation. 2007 Dec 11;116(24):2878-91
references.pmid: 18025533
references.citation: J Cardiovasc Magn Reson. 2007;9(5):815-21
references.pmid: 17891620
references.citation: Am J Cardiol. 2005 Dec 1;96(11):1568-70
references.pmid: 16310442
references.citation: J Am Coll Cardiol. 2004 Dec 21;44(12):2383-9
references.pmid: 15607402
references.citation: Magn Reson Imaging. 2004 Feb;22(2):155-61
references.pmid: 15010107
references.citation: Radiology. 2001 Jan;218(1):215-23
references.pmid: 11152805
references.citation: J Cardiovasc Magn Reson. 2006;8(4):661-70
references.pmid: 16869317