Early Postoperative Triple Targeted Therapy in Patients With CHD-PAH in the "Grey Zone"

This study is a prospective randomized controlled study. Including congenital heart disease combined with pulmonary hypertension, the hemodynamics in the right heart catheter before surgery conform to the "gray zone standard" (PVRi: 6-8 Wood \* m2) in the 2018 Cologne Consensus.PVR/SVR 0.3-05), acute vascular reaction test met the positive standard (PVRi: \< 6 Wood\*m 2, PVR/SVR \< 0.3), and CHD patients (including VSD, PDA, ASD) who underwent surgical repair, patients were randomly assigned to different treatment groups, The specific grouping is as follows: 1) 15 patients in the standard dual therapy group received dual therapy for 12 months, consisting of a phosphodiesterase type 5 inhibitor (tadalafil 20 mg qd or sildenafil 20\~25 mg tid) combined with a phosphodiesterase 5 receptor antagonist (amlodipine 5 mg qd or macitentan 10 mg qd). 2) 15 patients in the early triple therapy group received the combination of a phosphodiesterase type 5 inhibitor (tadalafil 20 mg qd or sildenafil 20\~25 mg tid), a phosphodiesterase-5 receptor antagonist (amlodipine 5 mg qd or maropitant 10 mg qd), and treprostinil. The dosage of pralatrexate needs to be titrated gradually, starting from 1.25 ng/min/kg, and titrated gradually to the patient's maximum tolerated dose (guideline dose of 15-20 ng/min/kg), and the duration of triple targeted drug therapy is at least 6 months. After 6 months, it can be adjusted to the standard dual therapy group to 12 months. Observation indexes The main observation indices are as follows:(1) Pulmonary vascular resistance measured by right heart catheterization one year after surgical repair and closure of defects. Secondary observation indicators: (1) Right atrial pressure, pulmonary artery pressure, PVR/SVR, cardiac index, mixed blood oxygen saturation, and systemic arterial blood oxygen saturation measured in right heart catheterization 12 months after surgery. (2) NT-proBNP level (3) 6-minute walk distance (4) WHO functional class (5) SF-36 self-assessment quality of life score (6) rehospitalization due to worsening heart failure, atrial septal defect shunt, lung transplant or heart-lung transplant, and other adverse events; (7) Drug-related adverse reaction events, including but not limited to hypotension, headache, muscle pain, injection site pain, injection site infection, bleeding, etc.