PMID- 20394877 OWN - NLM STAT- MEDLINE DCOM- 20100615 LR - 20211020 IS - 1558-3597 (Electronic) IS - 0735-1097 (Print) IS - 0735-1097 (Linking) VI - 55 IP - 16 DP - 2010 Apr 20 TI - Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation. PG - 1721-8 LID - 10.1016/j.jacc.2009.12.036 [doi] AB - 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/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. CI - Copyright (c) 2010 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved. FAU - Rathod, Rahul H AU - Rathod RH AD - Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA. FAU - Prakash, Ashwin AU - Prakash A FAU - Powell, Andrew J AU - Powell AJ FAU - Geva, Tal AU - Geva T LA - eng GR - T32 HL007572/HL/NHLBI NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - United States TA - J Am Coll Cardiol JT - Journal of the American College of Cardiology JID - 8301365 RN - 0 (Contrast Media) RN - K2I13DR72L (Gadolinium DTPA) SB - IM MH - Contrast Media/administration & dosage MH - Electrocardiography MH - Endomyocardial Fibrosis/complications/*diagnosis/physiopathology MH - Female MH - Follow-Up Studies MH - Fontan Procedure/*adverse effects MH - *Gadolinium DTPA/administration & dosage MH - Heart Defects, Congenital/surgery MH - Heart Ventricles/pathology/physiopathology MH - Humans MH - Injections, Intravenous MH - Magnetic Resonance Imaging, Cine/*methods MH - Male MH - Myocardial Contraction/physiology MH - Myocardium/*pathology MH - Prognosis MH - Retrospective Studies MH - Stroke Volume/physiology MH - Tachycardia, Ventricular/diagnosis/*etiology/physiopathology MH - Time Factors MH - Ventricular Dysfunction/diagnosis/*etiology/physiopathology MH - Young Adult PMC - PMC4266480 MID - NIHMS647132 EDAT- 2010/04/17 06:00 MHDA- 2010/06/16 06:00 PMCR- 2014/12/15 CRDT- 2010/04/17 06:00 PHST- 2009/09/22 00:00 [received] PHST- 2009/11/23 00:00 [revised] PHST- 2009/12/21 00:00 [accepted] PHST- 2010/04/17 06:00 [entrez] PHST- 2010/04/17 06:00 [pubmed] PHST- 2010/06/16 06:00 [medline] PHST- 2014/12/15 00:00 [pmc-release] AID - S0735-1097(10)00597-8 [pii] AID - 10.1016/j.jacc.2009.12.036 [doi] PST - ppublish SO - J Am Coll Cardiol. 2010 Apr 20;55(16):1721-8. doi: 10.1016/j.jacc.2009.12.036.