Conditions: Congenital Disorders
Keywords: QT Prolongation QT Interval Epinephrine Stress test
Allocation: NA | Intervention model: SINGLE_GROUP
Enrollment: 7
Healthy volunteers: no

Brief summary:
Long QT syndrome (LQTS)is a cardiac disorder that may lead to ventricular arrythmias and culminate in syncope and/or possible death.

Recently, researchers have developed a way of discovering patients with LQTS by using low doses of epinephrine by a continuous, intravenous infusion in adults. Epinephrine, or adrenaline, is produced by our bodies in times of stress. By producing adrenaline, your body allows itself to adapt to its stressful environment and take appropriate actions (i.e. fight or flight response). By simulating this response with very small amounts of epinephrine, researchers have shown prolongation of the QT interval does not occur in normal healthy adults. However, adults with confirmed LQTS Type 1 (LQTS-1) will prolong their QT interval when given low dose epinephrine. Therefore, this test can act as a safe means of identifying adults with LQTS-1 who do not have prolonged QT intervals on their resting EKGs.

However, LQTS is not just a disease of adults, it affects children as well. Currently the standard of care is to obtain resting EKGs on our pediatric patients which can miss those patients with concealed LQTS. Those patients, who are old enough, can undergo exercise testing. Yet this leaves young children unable to run on a treadmill without a diagnostic test.

Hypothesis: The low-dose epinephrine infusion stress test does not cause prolongation of the QT interval in an electrophysiologically normal healthy pediatric population.

Detailed description:
This study will involve asking fifty children coming to the hospital for their standard of care procedure to treat a separate cardiac condition to participate. Patients approached will already have an intravenous line in place for their procedure. After consent and assent as necessary are obtained, the patient be brought to a separate room for the low-dose epinephrine infusion trial. The patient will lie down on a table, be hooked up to an EKG machine and the infusion will start. The amount of epinephrine actually infused is based on the weight of the patient but is substantially less than the dose received from an epinephrine auto-injection for anaphylaxis. After about 40 minutes, the trial will be completed and the patient may return to the waiting room for their original procedure. We have performed this test on a number of pediatric patients suspected of having LQTS with promising results and with little to no side effects. The patient will be awake for the procedure and will be able to verbalize concerns or complaints. A pediatric electrophysiologist and nurse will be present throughout the entire procedure.

The purpose of this study is to determine if children who are infused with low amounts of epinephrine will also maintain their normal QT interval as it does with healthy adults. This study would hope to prove that low-dose epinephrine infusion trials can serve as a way of identifying children with LQTS so that they may be effectively treated and potentially have their lives saved.

Arms:
arm 1: Healthy pediatric subjects will receive epinephrine. Epinephrine infusion will begin at 0.025 ug/kg/minute, for ten minutes.The epinephrine infusion will then be increased to 0.05 ug/kg/minute for five minutes. The epinephrine infusion will then be increased to a maximal dose of 0.1 ug/kg/minute for five minutes. The epinephrine infusion is then discontinued.

Interventions: intervention 1: Epinephrine
Intervention descriptions: intervention 1: We will follow the protocol outlined by Ackerman et al. known as the Mayo protocol4 which is currently used routinely in our electrophysiology laboratory. Epinephrine infusion will begin at 0.025 ug/kg/minute, for ten minutes and a twelve-lead electrocardiogram is obtained at time=0 minutes, 5 minutes, and 10 minutes. The epinephrine infusion will then be increased to 0.05 ug/kg/minute for five minutes and the electrocardiogram repeated. The epinephrine infusion will then be increased to a maximal dose of 0.1 ug/kg/minute for five minutes and electrocardiogram obtained. The epinephrine infusion is then discontinued and measurements are obtained at five minutes and ten minutes after stopping the epinephrine infusion. The total time of procedure will be 35 minutes.

Protocol document: