Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation.
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.
Rathod
Rahul H
RH
Department of Cardiology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA.
Prakash
Ashwin
A
Powell
Andrew J
AJ
Geva
Tal
T
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
D003287:Contrast Media; D019786:Gadolinium DTPA
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