PMID- 17056346 OWN - NLM STAT- MEDLINE DCOM- 20061205 LR - 20151119 IS - 0002-9149 (Print) IS - 0002-9149 (Linking) VI - 98 IP - 9 DP - 2006 Nov 1 TI - 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. PG - 1277-82 AB - 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. FAU - Giardini, Alessandro AU - Giardini A AD - Pediatric Cardiology and Adult Congenital Unit, University of Bologna, Bologna, Italy. alessandro5574@iol.it FAU - Lovato, Luigi AU - Lovato L FAU - Donti, Andrea AU - Donti A FAU - Formigari, Roberto AU - Formigari R FAU - Oppido, Guido AU - Oppido G FAU - Gargiulo, Gaetano AU - Gargiulo G FAU - Picchio, Fernando Maria AU - Picchio FM FAU - Fattori, Rossella AU - Fattori R LA - eng PT - Journal Article DEP - 20060914 PL - United States TA - Am J Cardiol JT - The American journal of cardiology JID - 0207277 RN - 0 (Contrast Media) RN - AU0V1LM3JT (Gadolinium) SB - IM MH - Adolescent MH - Adult MH - Age Factors MH - *Cardiac Surgical Procedures MH - Contrast Media MH - Exercise Test MH - Exercise Tolerance MH - Female MH - Gadolinium MH - Heart Failure/epidemiology/etiology/physiopathology MH - Heart Ventricles/*abnormalities/physiopathology/surgery MH - Humans MH - Image Enhancement MH - Magnetic Resonance Imaging/methods MH - Male MH - Middle Aged MH - Observer Variation MH - Oxygen Consumption MH - Prevalence MH - Stroke Volume MH - Transposition of Great Vessels/epidemiology/pathology/*physiopathology/*surgery MH - Treatment Outcome MH - Ventricular Function, Right EDAT- 2006/10/24 09:00 MHDA- 2006/12/09 09:00 CRDT- 2006/10/24 09:00 PHST- 2006/02/01 00:00 [received] PHST- 2006/05/24 00:00 [revised] PHST- 2006/05/24 00:00 [accepted] PHST- 2006/10/24 09:00 [pubmed] PHST- 2006/12/09 09:00 [medline] PHST- 2006/10/24 09:00 [entrez] AID - S0002-9149(06)01381-6 [pii] AID - 10.1016/j.amjcard.2006.05.062 [doi] PST - ppublish SO - Am J Cardiol. 2006 Nov 1;98(9):1277-82. doi: 10.1016/j.amjcard.2006.05.062. Epub 2006 Sep 14.