Pulmonary arterial hypertension
Conditions
Interventions
Dual targeted drug therapy group:Nil
Triple targeted drug therapy group:Nil
Sponsors
Shanghai Pulmonary Hospital
Eligibility
Sex/Gender
All
Age
14 Years to 80 Years
Inclusion criteria
Inclusion criteria: 1.Aged 14 to 80 years; 2.It is diagnosed as PAH in the first category of PAH clinical classification updated by WHO and belongs to one of the following subgroups; (1)Idiopathic pulmonary hypertension; (2)Hereditary pulmonary hypertension; (3)Drug or poison-related pulmonary hypertension; (4)Pulmonary hypertension related to connective tissue diseases; (5)Pulmonary hypertension related to congenital heart disease. 3.The right heart catheterization has been performed in the past, and it meets the PAH right heart catheterization hemodynamic diagnostic criteria: Mean pulmonary artery pressure (mPAP) >= 25 mm Hg, pulmonary arteriole pressure (PAWP) = 3 Wood units; 4.Dual targeted drug therapy (endothelin receptor antagonist + type 5 phosphodiesterase inhibitor/guanylate cyclase agonist) >= 6 months; 5.REVEAL2.0 score > 6 points; 6.Sign the written informed consent; 7.Women of childbearing age use contraceptive therapy.
Exclusion criteria
Exclusion criteria: 1.Participated in other clinical trials or are participating in other clinical trials within 3 months before being selected; 2.Other types of pulmonary hypertension (Patients in categories 2, 3, 4, and 5 of the PH clinical classification updated by WHO); 3.The patient was unable to cooperate with the investigator during the trial; 4.In high-risk patients, intravenous or subcutaneous injection of Treprostinil is preferred, and the patient agrees to use Treprostinil for treatment. 5.Serum alanine aminotransferase (ALT) or aspartate aminotransferase (AST) level> 2 times ULN; 6.Severe liver insufficiency (Child-Pugh grade C); 7.Severe renal insufficiency (creatinine clearance rate 50ml); 12.Combined with active tumor; 13.Patients started receiving regular rehabilitation exercise prescriptions within 3 months before being selected; 14.Pregnant or lactating women; 15.Obvious obstructive or restrictive lung disease, recent lung function: total lung volume <70% of normal expected value; forced expiratory volume in the first second (FEV1) <60% of normal expected value; forced expiratory volume/strength in one second Vital capacity ratio (FEV1/FVC) <65%; 16.Patients considered by other researchers to be unsuitable to participate in this study.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| REVEAL 2.0 score; | — |
Secondary
| Measure | Time frame |
|---|---|
| Six minutes walking distance;Quality of life score;Plasma NT proBNP level;Echocardiography to estimate right ventricular systolic function;Time to clinical worsening events;World Health Organization functional classification; | — |
Countries
China
Contacts
Public ContactZhao Qinhua
Shanghai Pulmonary Hospital
Outcome results
None listed