Relation Between Right Ventricular Structural Alterations and Markers of Adverse Clinical Outcome in Adults With Systemic Right Ventricle and Either Congenital Complete (After Senning Operation) or Congenitally Corrected Transposition of the Great Arteries

Giardini, Alessandro; Lovato, Luigi; Donti, Andrea; Formigari, Roberto; Oppido, Guido; Gargiulo, Gaetano; Picchio, Fernando Maria; Fattori, Rossella

doi:10.1016/j.amjcard.2006.05.062

Cited by 109 publications

(82 citation statements)

References 28 publications

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“…17 In the systemic left ventricle (LV), LGE has prognostic significance in ischemic heart disease and cardiomyopathies. [18][19][20][21] Previous studies of RV LGE in patients with a systemic RV showed that its presence and extent relate to impaired RV function, 1,22 exercise intolerance, 22 and arrhythmia. 1,22 These early studies, however, were purely cross-sectional and lacked histological validation.…”

Section: Rydman Et Al Systemic Rv Lge and Cardiac Events In Tgamentioning

confidence: 99%

“…[18][19][20][21] Previous studies of RV LGE in patients with a systemic RV showed that its presence and extent relate to impaired RV function, 1,22 exercise intolerance, 22 and arrhythmia. 1,22 These early studies, however, were purely cross-sectional and lacked histological validation. 1,22 We hypothesized that fibrosis detected by RV LGE would predict adverse outcome in a prospective study of these patients.…”

Section: Rydman Et Al Systemic Rv Lge and Cardiac Events In Tgamentioning

confidence: 99%

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Systemic Right Ventricular Fibrosis Detected by Cardiovascular Magnetic Resonance Is Associated With Clinical Outcome, Mainly New-Onset Atrial Arrhythmia, in Patients After Atrial Redirection Surgery for Transposition of the Great Arteries

Rydman

Gatzoulis

et al. 2015

Circ: Cardiovascular Imaging

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M yocardial fibrosis is implicated in late right ventricular (RV) dysfunction in patients who have undergone atrial redirection surgery (Mustard/Senning operation) for transposition of the great arteries.1,2 The RV in these patients functions as the systemic ventricle which predisposes to arrhythmia, premature systemic RV failure, and sudden death.3-13 Atrial tachyarrhythmia has been reported as the most common early clinical complication, 5,7,11,13,14 is associated with significant clinical morbidity, has been suggested to reflect systemic RV dysfunction, and is a recognized marker of elevated atrial pressure and increased mortality. 5,7,13,15 Thereafter, RV dysfunction precedes the onset of symptoms, clinical heart Background-We hypothesized that fibrosis detected by late gadolinium enhancement (LGE) cardiovascular magnetic resonance predicts outcomes in patients with transposition of the great arteries post atrial redirection surgery. These patients have a systemic right ventricle (RV) and are at risk of arrhythmia, premature RV failure, and sudden death. Methods and Results-Fifty-five patients (aged 27±7 years) underwent LGE cardiovascular magnetic resonance and were followed for a median 7.8 (interquartile range, 3.8-9.6) years in a prospective single-center cohort study. RVLGE was present in 31 (56%) patients. The prespecified composite clinical end point comprised new-onset sustained tachyarrhythmia (atrial/ventricular) or decompensated heart failure admission/transplantation/death. Univariate predictors of the composite end point (n=22 patients; 19 atrial/2 ventricular tachyarrhythmia, 1 death) included RV LGE presence and extent, RV volumes/mass/ejection fraction, right atrial area, peak Vo 2 , and age at repair. In bivariate analysis, RVLGE presence was independently associated with the composite end point (hazard ratio, 4.95 [95% confidence interval, 1.60-15.28]; P=0.005), and only percent predicted peak Vo 2 remained significantly associated with cardiac events after controlling for RV LGE (hazard ratio, 0.80 [95% confidence interval, 0.68-0.95]; P=0.009/5%). In 8 of 9 patients with >1 event, atrial tachyarrhythmia, itself a known risk factor for mortality, occurred first. There was agreement between location and extent of RV LGE at in vivo cardiovascular magnetic resonance and histologically documented focal RV fibrosis in an explanted heart. There was RV LGE progression in a different case restudied for clinical indications. Conclusions-Systemic RV LGE is strongly associated with adverse clinical outcome especially arrhythmia in transposition of the great arteries, thus LGE cardiovascular magnetic resonance should be incorporated in risk stratification of these patients. (Circ Cardiovasc Imaging. 2015;8:e002628.

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Section: Rydman Et Al Systemic Rv Lge and Cardiac Events In Tgamentioning

confidence: 99%

Section: Rydman Et Al Systemic Rv Lge and Cardiac Events In Tgamentioning

confidence: 99%

Systemic Right Ventricular Fibrosis Detected by Cardiovascular Magnetic Resonance Is Associated With Clinical Outcome, Mainly New-Onset Atrial Arrhythmia, in Patients After Atrial Redirection Surgery for Transposition of the Great Arteries

Rydman

Gatzoulis

et al. 2015

Circ: Cardiovascular Imaging

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“…14) It should be emphasized that all of these previously discussed patients with ccTGA were much younger (mean age, 20-38 years) compared to our patient, 2,[12][13][14] where we clearly detected extensive, transmural myocardial scars (presumably fibrosis) associated with severely impaired systemic RV function and enlarged volumes using CMR, in an elderly patient with uncomplicated ccTGA and situs inversus for the first time. Therefore, this case may provide new insights into the natural history of this population with such a long-life and the association between systemic RV failure and myocardial fibrosis, especially in an elderly patient.…”

Section: Discussionmentioning

confidence: 96%

Congenitally Corrected Transposition of the Great Arteries and Situs Inversus in an Octogenarian With Systemic Right Ventricular Failure

et al. 2017

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SummarySystemic right ventricular (RV) failure in patients with congenitally corrected transposition of the great arteries (ccTGA), a major cause of mortality in the long-term follow-up, is usually induced by concomitant severe morphologically tricuspid regurgitation (TR) with/without Ebstein's anomaly or progressive conduction tissue disturbances. However, whether or not myocardial fibrosis is a common cause of systemic RV failure in patients with ccTGA remains unclear. Here, we describe an 82-year-old man who had been diagnosed previously as having uncomplicated ccTGA and situs inversus and recently developed systemic RV failure, which was neither associated with severe TR nor advanced conduction tissue abnormalities. Cardiovascular magnetic resonance (CMR) with delayed-enhancement imaging clearly detected extensive myocardial scars (presumably fibrosis) in the RV wall as well as prominent dilatation, hypertrophy, and systolic dysfunction of the systemic RV. These findings suggest that myocardial fibrosis can cause systemic RV failure in elderly patients with uncomplicated ccTGA despite the absence of severe TR or advanced conduction tissue abnormalities and that CMR may be a useful examination to accurately detect systemic RV failure associated with myocardial fibrosis and to subsequently clarify the prognosis in these patients. (Int Heart J 2017; 58: 151-154)

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“…However, the presence of LGE in patients with a systemic RV is associated with RV dysfunction, poor exercise tolerance, arrhythmia, and progressive clinical deterioration. 40 With these components of the imaging focus in mind, here is a suggested imaging protocol for adults with c-TGA:…”

Section: Myocardial Fibrosismentioning

confidence: 99%

Cardiac Magnetic Resonance in Adults with Congenital Heart Disease

Partington

Valente

2013

Methodist DeBakey Cardiovascular Journal

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Increasing numbers of adults with congenital heart disease are referred for cardiac magnetic resonance imaging. Knowledge of the congenital heart anatomy, prior surgical interventions, and the development of an imaging focus for each individual patient plays a crucial role when performing a successful cardiac magnetic resonance imaging examination. The following manuscript focuses on cardiac magnetic resonance imaging considerations of three specific conotruncal congenital heart lesions: tetralogy of Fallot, transposition of the great arteries (TGA), and physiologically corrected TGA (c-TGA).

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Relation Between Right Ventricular Structural Alterations and Markers of Adverse Clinical Outcome in Adults With Systemic Right Ventricle and Either Congenital Complete (After Senning Operation) or Congenitally Corrected Transposition of the Great Arteries

Cited by 109 publications

References 28 publications

Systemic Right Ventricular Fibrosis Detected by Cardiovascular Magnetic Resonance Is Associated With Clinical Outcome, Mainly New-Onset Atrial Arrhythmia, in Patients After Atrial Redirection Surgery for Transposition of the Great Arteries

Systemic Right Ventricular Fibrosis Detected by Cardiovascular Magnetic Resonance Is Associated With Clinical Outcome, Mainly New-Onset Atrial Arrhythmia, in Patients After Atrial Redirection Surgery for Transposition of the Great Arteries

Congenitally Corrected Transposition of the Great Arteries and Situs Inversus in an Octogenarian With Systemic Right Ventricular Failure

Cardiac Magnetic Resonance in Adults with Congenital Heart Disease

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