Heart Defects, Congenital, Tricuspid Atresia
Conditions
Keywords
cardiovascular and respiratory diseases, heart defects, rare disease
Brief summary
OBJECTIVES: I. Determine the pharmacokinetics of exogenous liothyronine administered in children undergoing the modified Fontan procedure. II. Determine the liothyronine supplementation dose that counters the fall in serum liothyronine concentrations and provides the greatest potential myocardial benefit after the modified Fontan procedure. III. Evaluate the potential toxicity of exogenous liothyronine administered in children undergoing a modified Fontan procedure.
Detailed description
PROTOCOL OUTLINE: This is a randomized, placebo controlled, dose escalation study. Initially, patients are randomized to receive either 1 of 3 different dosages of liothyronine or placebo after undergoing the Fontan procedure. If no unacceptable toxicity is observed in this group, a third dose level of liothyronine is added to the randomization. A total of 7 patients are enrolled at each dose level. All randomized study drugs are administered by continuous infusion over 1 hour after surgery. Cardiac function is assessed 5 days after surgery.
Interventions
DRUGliothyronine I 131
Sponsors
Children's Hospital and Health Center
FDA Office of Orphan Products Development
Study design
Allocation
RANDOMIZED
Primary purpose
TREATMENT
Eligibility
Sex/Gender
ALL
Age
No minimum to 17 Years
Healthy volunteers
No
Inclusion criteria
* Single ventricle congenital cardiac malformation * Must undergo modified Fontan procedure * No concurrent medications known to interfere with thyroid metabolism including propranolol and amiodarone * No hepatic dysfunction * No renal dysfunction * No pre-existing thyroid dysfunction
Outcome results
None listed