Pulmonary Hypertension Associated with Congenital Heart Disease
Conditions
Interventions
Trial Group (Part One):Plasma samples of children aged 0-14 years with shunt CHD-PAH were prospectively collected for non-targeted metabolomics sequencing analysis to search for metabolic differential
Control Group (Part One):Plasma samples of control children were prospectively collected for non-targeted metabolomics sequencing analysis to search for metabolic differential markers.
Experimental Group (Part Two):iNO 20 ppm + IV Treprostinil 30 ng/(kg·min)
Control Group (Part Two):iNO 20 ppm
Sponsors
Fuwai Hospital, Chinese Academy of Medical Sciences
Eligibility
Sex/Gender
All
Age
No minimum to 14 Years
Inclusion criteria
Inclusion criteria: Part I Metabolomic Characterization of CHD-PAH 1.Case group: (1) Shunt-type CHD patients aged 0–14 years with severe PAH (mean pulmonary arterial pressure > 50 mmHg on echocardiography or right-heart catheterization): including ventricular septal defect, atrial septal defect, patent ductus arteriosus, endocardial cushion defect, aortopulmonary window, total anomalous pulmonary venous return, transposition of the great arteries, double-outlet right ventricle, and truncus arteriosus. (2) Written informed consent obtained from the legal guardian. 2.Control group: CHD children undergoing surgery during the same period whose pulmonary arterial pressure was documented as normal. Part II Optimized Pulmonary Vascular-Targeted Pharmacologic Strategy to Reduce the Risk of Post-operative Pulmonary Hypertensive Crisis in Pediatric CHD 1.Post-operative shunt-type CHD patients aged 0–14 years: including ventricular septal defect, atrial septal defect, patent ductus arteriosus, endocardial cushion defect, aortopulmonary window, total anomalous pulmonary venous return, transposition of the great arteries, double-outlet right ventricle, and truncus arteriosus. 2,Concurrent severe PAH (pre-operative echocardiography or right-heart catheterization confirming severe PAH, plus post-operative pressure ratio PAPs/ABPs > 60 %). 3.Written informed consent obtained from the legal guardian.
Exclusion criteria
Exclusion criteria: Part I Metabolomic Characterization of CHD-PAH 1.Pulmonary hypertension due to left-heart disease, single-ventricle physiology, or segmental PAH. 2.Eisenmenger syndrome. 3.Post-operative ECMO support. 4.Concurrent extra-cardiac disorders that alter metabolism: inborn metabolic errors, chromosomal translocation syndromes, autoimmune diseases, etc. 5.Refusal of follow-up or biospecimen collection. Part II Optimized Pulmonary Vascular-Targeted Pharmacologic Strategy to Reduce Post-operative Pulmonary Hypertensive Crisis in Pediatric CHD 1.Pulmonary hypertension attributable to left-heart disease: hypoplastic left-heart syndrome, mitral stenosis, aortic stenosis, pulmonary-vein stenosis. 2.Requirement for post-operative ECMO. 3.Creation of an ASD/VSD fenestration.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Incidence of Pulmonary Hypertensive Crisis; | — |
Secondary
| Measure | Time frame |
|---|---|
| Pulmonary Artery Systolic Pressure to Systemic Arterial Systolic Pressure (PAPs/ABPs);Mean Arterial Pressure;Central Venous Pressure;Vasoactive-Inotropic Score;Use of ECMO;Mechanical ventilation duration;ICU length of stay;Total hospital length of stay;Total hospitalization costs; | — |
Countries
China
Contacts
Public ContactWang Xu
Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College
Outcome results
None listed