CHD-PAH (pulmonary arterial hypertension with congenital heart disease)
Conditions
Interventions
None listed
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: 1. Any age 2. PH with any CHD who have no plan to have cardiac surgery or catheter intervention within 6 months 3. The diagnosis of PAH is made by cardiac catheterization. mean PAp>20mmHg or PVRI>=3Woodm2 4. Both cases with repaired or unrepaired shunt lesion 5. Cases with Fontan circulation or complex heart disease with major aortopulmonary collateral arteries included as specific patient groups 6. Cases participating another study
Exclusion criteria
Exclusion criteria: 1. A case planned to have cardiac surgery or catheter intervention within 6 months 2. A case performed cardiac surgery or catheter intervention within 6 months 3. A case with extremely unstable status 4. A case without informed consent 5. A case judged as inappropriate by principal investigator
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Survival rate of CHD-PAH patients | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Status of medical treatment in CHD-PAH patients 2. Factors influencing outcome (survival rate) 3. Predictive factors of clinical worsening 4. Relationship between treatment drugs and outcome 5. Validity of treat and repair strategy 6. Similarity and difference of clinical course according to the shunt sites | — |
Countries
Japan
Contacts
Institute of Science Tokyo Department of Pediatrics