However, even modest residual levels of PAH substantially determine intermediate and longer-term out- comes. Patients should be followed for pulmo- nary hypertension. 5. Although history, noninvasive testing, and labo- ratory analysis (biochemistry and hematology) are all part of the workup of pulmonary hyperten- sion associated with CHD, cardiac catheterization with careful hemodynamic measurements, with or without provocative maneuvers and/or angiog- raphy, remains fundamental to accurate diagnosis and design of therapeutic plans. 4.4.6.2. Eisenmenger Syndrome Recommendations for Eisenmenger Syndrome Referenced studies that support recommendations are summarized in Online Data Supplement 50. COR LOE Recommendations Diagnostic I C-EO When evaluating adults with presumed Eisenmenger syndrome, clinicians should confirm diagnostic imaging and cardiac catheterization data accuracy and exclude other potential contributors to right-to-left shunting or pulmonary hypertension. Therapeutic I A Bosentan is beneficial in symptomatic adults with Eisenmenger syndrome with ASD or IIa B-R In symptomatic adults with Eisenmenger syndrome, bosentan and PDE-5 inhibitors are reasonable in combination if symptomatic improvement does not occur with either IIa C-EO Bosentan is a reasonable therapy to treat symptomatic adults with Eisenmenger syndrome with 1 of the following: shunts other than ASD/VSD (eg, PDA, aortopulmonary window) (Level of Evidence C-EO), or complex congenital or Down (Level of Evidence B-NR). B-NR IIa B-NR It is reasonable to use PDE-5 inhibitors (eg, sildenafil, tadalafil) to treat symptomatic adults with Eisenmenger syndrome with ASD, VSD, or great artery Synopsis Historically Eisenmenger syndrome has been under- stood as the most advanced form of PAH associated with congenital intracardiac and great arterial shunting. The natural course and outcomes of PAH in patients with ACHD with Eisenmenger syndrome, as contrasted to other adults with PAH, remain incompletely defined. 