Efficacy of Triple-Combination Therapy in Severe PAH-CHD

Inclusion Criteria: 1. Subject is diagnosed with congenital heart diseases (CHD) and associated pulmonary arterial hypertension (PAH) 2. Subject is under borderline hemodynamics status and ineligible for congenital heart diseases closure, confirmed by RHC: Qp/Qs：\< 1.5, Rp/Rs \> 0.3 and PVR \> 5 Wood units 3. Subject signs an informed Consent Form and is willing to participate in follow-up visits Exclusion Criteria: 1. Subject is diagnosed with other etiology of pulmonary arterial hypertension, e.g. left heart diseases associated pulmonary arterial hypertension. 2. Subject is diagnosed with other types of PAH-CHD, e.g. Eisenmenger syndrome, PAH with small/coincidentalb defects 3. Subject had cerebral hemorrhage, bleeding events in other organs within 3 months or was in high risk of bleeding. 4. Subject is diagnosed of hepatic insufficiency: ALT or AST\>3×ULN at the screening visit. 5. Subject is diagnosed of moderate to severe renal insufficiency: eGFR\<30ml/min/1.73m2 at the screening visit. 6. Subject has uncontrolled arrhythmia with clinical significance within 90 days. 7. Subject is diagnosed of the following diseases or received following medical interventions with 90 days: unstable angina, severe coronary atherosclerosis or myocardial infarction, cerebrovascular disease, deep vein thrombosis, pulmonary embolism, percutaneous coronary intervention, coronary artery bypass grafting, carotid artery intervention, peripheral artery intervention. 8. Subject is diagnosed of malignant tumor (exception: tumors that have been cured and have not recurred in the last 5 years, basal cell and squamous cell skin cancers that have been completely resected, and cancers of any type in situ that have been completely resected) 9. Subject cannot follow the study procedure due to other acute or chronic diseases. 10. Subject is under other RCT. 11. Subject has a life expectancy \<1 year. 12. Subject cannot follow the study procedure due to other reasons in the opinion of the investigators. (alcoholic, drug abuse, lack of compliance)