Aberrations in Carnitine Homeostasis in Congenital Heart Disease With Increased Pulmonary Blood Flow (L-carn)
Heart Septal Defects, Ventricular, Atrioventricular Septal Defect
About this trial
This is an interventional treatment trial for Heart Septal Defects, Ventricular focused on measuring congenital heart disease, ventricular septal defect, atrioventricular septal defect, increased pulmonary blood flow, nitric oxide
Eligibility Criteria
Inclusion Criteria:
- have unrestrictive VSD, AVSD
- are undergoing complete repair
- are between 2-12 months of age
- are corrected gestational age ≥34 weeks
- will have an indwelling arterial or venous line
- have not had enteral or parenteral nutrition for at least 6 hrs
Exclusion Criteria:
- have body weight < 2.0 kg
- pulmonary artery or vein abnormalities not being addressed surgically
- suspected or proven in-born error of metabolism
- have other major congenital abnormalities that affect the cardiopulmonary system
- are taking carnitine supplementation
Sites / Locations
- University of California San Francisco
Arms of the Study
Arm 1
Experimental
IV L-carnitine
L-carnitine (25, 50, or 100mg/kg IV) will be given, 30-60 minutes prior to the initiation of CPB, and a second dose ~2 hr. following separation from CPB (with a minimum of 4 hrs from initial dose). The first 5 subjects will receive 25 mg/kg, with an escalation of dose after each 5 subjects enrolled. The study drug will be brought to the operating room and administered over 5 minutes by the anesthesiologist after an IV has been placed. Prior to the administration of the study drug, and again 24 and 48 hrs after CPB, 3.0 ml of blood will be collected for determinations of carnitine levels (free, total, and acylcarnitine), mitochondrial function, ROS and bioavailable NO as described in Aim 3A. Additional blood (0.5-1.0 ml) will be obtained to determine carnitine levels before CPB, and then before and 0.5, 1.5, 3, 5, 9, 12, and 24h after the second dose.