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Management of Adults With Congenital Heart Disease

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27 Evidence for Use of Risk Assessment Factors/Tools Evidence for Use of PAH-Specific Therapy Patient age, pretricuspid shunt, resting oxygen saturation, loss of sinus rhythm, pericardial effusion, BNP, C-reactive protein, 6MWD, echocardiography, renal dysfunction, or other clinical stratification tools.* Endothelin-receptor antagonists, phosphodiesterase-5 inhibitors, prostacylin analogs † Review using the 3-strata risk-prediction model for newly diagnosed patients, the 4-strata risk-prediction model for patients with established PAH, and/or any of the Eisenmenger syndrome tools. ‡ No direct studies on this isolated cohort; treatment has typically followed recommendations for idiopathic PAH Review using the 3-strata risk-prediction model for newly diagnosed patients, the 4-strata risk-prediction model for patients with established PAH, and/or any of the Eisenmenger syndrome tools. ‡ No direct studies on this isolated cohort; treatment has typically followed recommendations for idiopathic PAH Review using the 3-strata risk-prediction model for newly diagnosed patients, the 4-strata risk-prediction model for patients with established PAH, and/or any of the Eisenmenger syndrome tools. ‡ Phosphodiesterase-5 inhibitors, endothelin receptor antagonists, oral prostacyclin receptor analog, activin signaling inhibitors §

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