*Combination therapy with bosentan and PDE-5 inhibitor, if symptomatic improvement does not occur with either alone. ACHD indicates adult congenital heart disease; AR, aortic regurgitation; IE, infective endocarditis; LV, left ventricular; PAH, pulmonary artery hypertension; PASP, pulmonary artery systolic pressure; PDE-5, phosphodiesterase type-5 inhibitors; PH, pulmonary hypertension; Qp:Qs, pulmonary systemic blood flow ratio; and VSD, ventricular septal defect. CLINICAL STATEMENTS AND GUIDELINES much lower 25-year survival (42%). Larger defects may be repaired but only in the absence of severe PAH and severely elevated pulmonary vascular re- sistance, the presence of which incurs a high peri- Life expectancy after VSD closure in an adult is not normal but has improved over the past 50 years. Transcatheter device occlusion of muscular and peri- membranous VSD is feasible, and trials have dem- onstrated a good safety and efficacy profile.S4.1.3-16, VSD in adults is most commonly either small, or large and associated with Eisenmenger syn- drome; therefore, data regarding optimal manage- ment of moderate VSD in adults are lacking because of relative infrequency of a hemodynamically signif- icant VSD for which closure is an option. 2. Small restrictive defects of the muscular or mem- branous septum may be managed by observa- tion without need for operative intervention. However, 6% of patients with small supracristal (subaortic) or perimembranous defects may de- velop aortic valve prolapse and resultant AR that may be progressive.S4.1.3-1,S4.1.3-2,S4.1.3-18 There is a paucity of data supporting the timing of VSD clo- sure in patients with AR. Ideally, the VSD is closed if AR is progressive to avoid the continued wors- ening of AR and the need for aortic valve replace- ment. In the presence of a VSD, an aortic valve cusp (usually the right coronary cusp) may pro- lapse and partially or completely close the VSD, often with associated AR. 