ET1 Concentration, Metabolic Pathway Activation, and Pulmonary Blood Flow in Infants Undergoing Superior Cavo-Pulmonary Anastomosis

University of Colorado, Denver50 enrolled

Overview

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.

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.

Study Type

OBSERVATIONAL

Enrollment

Conditions

Single-ventricle Pulmonary Vascular Resistance Abnormality Superior Cavo-Pulmonary Anastomosis Endothelin

Eligibility

Sex: ALLMin age: 1 MonthMax age: 2 Years

Medical Language ↔ Plain English

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.

Outcomes

Primary Outcomes

Difference in ET1 concentration between controls, study patients at pre-SCPA catheterization, and study patients in the post-operative period.

Time frame: 48 hours post-operatively

Difference in metabolomics profile between controls, study patients at pre-SCPA catheterization, and study patients in the post-operative period.

Time frame: 48 hours post-operatively

Secondary Outcomes

Association between ET1 concentration at pre-SCPA catheterization in study patients and clinical metrics of pre-operative pulmonary adequacy.

Time frame: 48 hours post-operatively

Association between metabolomics profile in study patients and clinical metrics of pre-operative pulmonary adequacy.

Time frame: 48 hours post-operatively

ET1 Concentration, Metabolic Pathway Activation, and Pulmonary Blood Flow in Infants Undergoing Superior Cavo-Pulmonary Anastomosis

Overview

Conditions

Interventions

Eligibility

Locations (1)

Outcomes

Primary Outcomes

Secondary Outcomes

Central Contacts