title: 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.
abstract: This study sought to determine if areas of late gadolinium enhancement (LGE) would be present in adults with systemic right ventricles and if LGE would be associated with markers of adverse outcomes. Using gadolinium-enhanced magnetic resonance and cardiopulmonary exercise testing, 34 adults with systemic right ventricles (23 patients with atrial repair for transposition of the great arteries and 11 with congenitally corrected transposition) were studied at a mean age of 25 years. LGE was present in 14 patients (41%). The presence of LGE was associated with older age (p = 0.037), a lower right ventricular (RV) ejection fraction (34% vs 45%, p = 0.006), higher RV wall stress (p = 0.0001), reduced peak oxygen uptake (47% vs 56%, p = 0.001), and a history of arrhythmia (p = 0.005). The RV ejection fraction was correlated with RV wall stress (r = -0.81, p <0.0001) and peak oxygen uptake (r = 0.74, p <0.0001). Twelve patients experienced worsening of their clinical conditions. This was associated with decreases in biventricular function and increases in the prevalence and number of LGE areas. In conclusion, patients with systemic right ventricles have areas of abnormal myocardium, presumably due to fibrosis, that can be seen by contrast-enhanced magnetic resonance imaging. The presence of abnormal myocardial regions is associated with RV dysfunction, poor exercise tolerance, arrhythmia, and progressive clinical deterioration.
authors.affiliation: Pediatric Cardiology and Adult Congenital Unit, University of Bologna, Bologna, Italy. alessandro5574@iol.it
authors.forename: Alessandro
authors.initials: A
authors.lastname: Giardini
authors.forename: Luigi
authors.initials: L
authors.lastname: Lovato
authors.forename: Andrea
authors.initials: A
authors.lastname: Donti
authors.forename: Roberto
authors.initials: R
authors.lastname: Formigari
authors.forename: Guido
authors.initials: G
authors.lastname: Oppido
authors.forename: Gaetano
authors.initials: G
authors.lastname: Gargiulo
authors.forename: Fernando Maria
authors.initials: FM
authors.lastname: Picchio
authors.forename: Rossella
authors.initials: R
authors.lastname: Fattori
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D006348:Cardiac Surgical Procedures; D003287:Contrast Media; D005080:Exercise Test; D017079:Exercise Tolerance; D005260:Female; D005682:Gadolinium; D006333:Heart Failure / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D006352:Heart Ventricles / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D007089:Image Enhancement; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D010101:Oxygen Consumption; D015995:Prevalence; D013318:Stroke Volume; D014188:Transposition of Great Vessels / Q000453:epidemiology / Q000473:pathology / Q000503:physiopathology* / Q000601:surgery*; D016896:Treatment Outcome; D016278:Ventricular Function, Right
chemical_list: D003287:Contrast Media; D005682:Gadolinium