Study of the Relationship Between Calcium Levels and Intact Parathyroid Hormone (iPTH) in Adults With Repaired or Palliated Conotruncal Cardiac Defects

Hypoparathyroidism, Tetralogy of Fallot, Pulmonary Valve Stenosis, Conotruncal Cardiac Defects, Heart Defects, Congenital, Pulmonary Atresia

DiGeorge syndrome, cardiovascular and respiratory diseases, conotruncal cardiac defects, endocrine disorders, genetic diseases and dysmorphic syndromes, hypoparathyroidism, pulmonary valve stenosis, rare disease, tetralogy of Fallot

OBJECTIVES: I. Identify latent hypoparathyroidism in normocalcemic adult survivors with repaired conotruncal cardiac defects, by evaluating parathyroid gland secretory function after induced hypocalcemia. II. Determine the relationship of parathyroid hormone secretion to microdeletions in the same region of chromosome 22q11 as found in patients with DiGeorge anomaly.

PROTOCOL OUTLINE: Patients are given sodium citrate over a 2 hour infusion on day 1. Blood is drawn at times -30, -15, 0, and every 10 minutes thereafter during the infusion. On day 2, patients are given calcium gluconate over a 2 hour infusion. Blood is drawn at times -30, -15, 0, and every 10 minutes thereafter during the infusion.

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