Pulmonary arterial hypertension associated with congenital heart disease
Conditions
Interventions
riociguat group:NA
Sponsors
Second Xiangya Hospital, Central South University
Eligibility
Sex/Gender
All
Age
18 Years to 75 Years
Inclusion criteria
Inclusion criteria: 1. Age: 18-75 years old 2. Diagnosis: right heart catheterization standard: mPAP >= 20mmHg, PAWP 2 wood 3. Have a history of congenital heart disease 4. Condition: stable for 3 months or more. Defined as stable WHO-FC, no heart failure, and no frequent dose increases (less than once a month) 5. Risk stratification: intermediate- and high-risk groups. According to the Chinese Guidelines for the Diagnosis and Treatment of Pulmonary Arterial Hypertension (2021 Edition), those with at least 3 low-risk indicators and no high-risk indicators are defined as low-risk states, those with at least 2 high-risk indicators (including cardiac index or SvO2) are defined as high-risk states, and those who do not meet the low-risk and high-risk conditions are considered intermediate-risk states 6. Treatment history: Stable treatment with PDE-5i (sildenafil/tadalafil) monotherapy, PDE-5i combined with double or multiple drugs other than riociguat and the treatment time > 3 months, and currently switching from sildenafil/tadalafil to levocetirizine treatment. 7. Women need birth control
Exclusion criteria
Exclusion criteria: 1. Pregnant or lactating women; 2. Patients with conditions, such as malignancy or orthopaedic disease, that may hinder their ability to participate or complete the study were assessed by the investigators. 3. Patients with cardiopulmonary complications; 4. Patients who changed or discontinued any targeted therapy for PAH within 30 days.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Six minutes walking distance; | — |
Secondary
| Measure | Time frame |
|---|---|
| NT-proBNP;WHO-FC;Borg dyspnea rating scale;Cardiac ultrasound; | — |
Countries
China
Contacts
Public ContactLi Jiang
Second Xiangya Hospital, Central South University
Outcome results
None listed