Objective: To evaluate the impact of new farmacological options in adults with PAH CHD-related. Methods: we enrolled between January 2001 - November 2008 all the patients with PAH CHD-related. Results: the patients were 65 (27 M, 38 F, mean age 38.4 ± 13.5). 22 had ventricular septal defect, 17 atrio-ventricular canal, 10 atrial septal defect, 7 single ventricle, 5 atresia of pulmonary valve with ventricular septal defect, 4 persistent ductus arteriosus. At the rest, the NYHA class was, III 47 for patients and IV for 18 patients. The O2 saturation at rest was 80.4±10.5%. The mean pulmonary pression was 76.4±21.6 mmHg; the mean pulmonary artery resistances was 8.7±4.8 Wood Unit/m2; the mean QP/QS was 0.9±0.5. Then, 30 patients are under therapy with oral bosentan (125 mg x 2 /day or 62.5 mg x 2 /day), 7 with oral sildenafil (20 mg x 3/day), 5 patients with oral sitaxentan (100 mg/day), 17 with oral bosentan and oral sildenafil, 2 with oral sitaxentan and oral sildenafil, 4 with oral bosentan, oral sildenafil and epoprostenol ev (9.5ng/kg/min). At the follow-up (mean 3,1±2,2yrs), the NYHA class was improved for 50/65 (77%) patients, the actual NYHA class is I for 7 patients, II for 31 patients, III for 21 patients and IV for 6 patients. Conclusion: A complex therapeutical management is required for patient with PAH CHD-related. In our experience the new farmacological treatments were safe and well tolerated in adults patients with PAH CHD-related; we observed an unexpected and significatevely improvement of NYHA class.