S4.4.1.4-10 addressed the role of medical therapies for manage- ment of functional deterioration in systemic RVsS4.4.1.4- (see additional details in Section 3.17). See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; Section 3.4 for rec- ommendations on diagnostic evaluation; and Table 31 for routine testing and follow-up intervals. Recommendation-Specific Supportive Text 1. CMR is useful for quantification of systemic RV size and function.S4.4.1.4-1,S4.4.1.4-2 Administration of gadolinium contrast is useful in identifying fi- brotic myocardium demonstrated by late gado- 2. Symptomatic adults with CCTGA and severe TR with no more than mildly depressed systemic ven- tricular function should be evaluated for tricuspid valve replacement. In general, tricuspid valve re- placement is preferred to tricuspid repair in the adult CCTGA population. TR is often because of a dysplastic tricuspid valve and has been shown to be an independent predictor of death in CCTGA Systemic RV dysfunction is often attributable to longstanding TR, and efforts should be made to relieve the TR before worsen- Tricuspid valve repair has been attempted; however, recurrent clinically significant TR is observed frequently after tricuspid valve repair in patients with CCTGA; hence, valve replacement is preferred.S4.4.1.4-15 3. Many adult CCTGA patients are referred for tri- cuspid valve replacement late, when symptomatic and already suffering from moderate-to-severe CLINICAL STATEMENTS AND GUIDELINES TR and ventricular dysfunction.S4.4.1.4-16 In CCTGA patients referred for TVR, 10-year postopera- tive survival is <20% when the preoperative sys- temic ventricular ejection fraction is <40%S4.4.1.4-9 In a retrospective review of 46 CCTGA patients referred for TR surgery, preoper- ative systemic ventricular ejection fraction was the only independent predictor of postoperative sys- temic ventricular ejection fraction at 1 year.S4.4.1.4-3 4. 