Endothelin, Metabolomics, Pulmonary Vascular Resistance Abnormality, Single-ventricle, Superior Cavo-Pulmonary Anastomosis
Conditions
Brief summary
This is a novel preliminary study of biomarkers of pathologic pre-operative pulmonary vascular development, elevated pre-operative Pulmonary Vascular Resistance Index (PVRi), and complications associated with decreased post-operative pulmonary blood flow in single ventricle patients undergoing superior cavo-pulmonary anastomosis (SCPA). The study will utilize a combined targeted and untargeted approach to both optimize translation of a promising existing biomarker and efficiently identify novel biomarkers and potential therapeutic targets in this population.
Detailed description
Overall Hypothesis: Endothelin-1 (ET1) and associated dysregulation of key metabolic pathways decrease pre-operative pulmonary blood vessel development and increase post-operative pulmonary blood vessel resistance leading to decreased pulmonary blood flow in patients undergoing SCPA. For enrolled patients, collected data will include: * 3 mL blood sample (x2) at pre-SCPA catheterization. * 3 mL blood samples at 2, 24, and 48 hours post-operative. * Urine sample pre-operatively and post-operatively * Collection of otherwise-discarded operative tissue sample from the pulmonary artery. * Collection of clinical data, demographic data, and results of routine, post-operative hemodynamic monitoring.
Interventions
OTHERResearch Blood Sampling
Blood samples will be collected at specified time points and research assays will be performed.
Sponsors
University of Colorado, Denver
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
1 Months to 2 Years
Inclusion criteria
* Congenital heart disease patients undergoing catheterization for pre-SPCA evaluation or undergoing SCPA without plans for cardiac catheterization (utilizing data from a previously performed clinical catheterization). * All patients will have age from 31 days to 2 years.
Exclusion criteria
* Patients who will remain post-op with a pulsatile source of pulmonary blood flow in addition to the cavo-pulmonary anastomosis (so called "1.5 ventricle" repair) will be excluded. * Due to limitations in acceptable sample blood volumes for research, patients with weight \<4kg will be excluded. * Patients will not be excluded on the basis of gender, ethnicity, genetic diagnosis, gestational age at birth, non-cardiac comorbidity, or pre-operative medication regimen.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Difference in ET1 concentration between controls, study patients at pre-SCPA catheterization, and study patients in the post-operative period. | 48 hours post-operatively |
| Difference in metabolomics profile between controls, study patients at pre-SCPA catheterization, and study patients in the post-operative period. | 48 hours post-operatively |
Secondary
| Measure | Time frame |
|---|---|
| Association between ET1 concentration at pre-SCPA catheterization in study patients and clinical metrics of pre-operative pulmonary adequacy. | 48 hours post-operatively |
| Association between metabolomics profile in study patients and clinical metrics of pre-operative pulmonary adequacy. | 48 hours post-operatively |
Countries
United States
Contacts
PRINCIPAL_INVESTIGATORBenjamin Frank, MD
University of Colorado, Denver
Outcome results
None listed