Diltiazem in Jervell and Lange-Nielsen Syndrome
试验速览
- 阶段
- 4 期
- 状态
- 已完成
- 入组人数
- 1
- 试验地点
- 1
- 主要终点
- QT Interval
研究概览
简要总结
This study will test the effect of diltiazem, a calcium channel blocking drug, on the QT interval in patients with Jervell and Lange-Nielsen syndrome. This will be a single IV dose and acute effects (within minutes) will be observed.
详细描述
Subjects will be recruited by study personnel and will be scheduled for a single outpatient visit. If an implanted cardiac device (pacemaker or ICD) is in place, it will be interrogated to ensure no recent rhythm abnormalities. Just prior to administering diltiazem, an ECG and BP will be obtained. If baseline SBP is < 100 mm Hg or DBP < 60 mm Hg, the study will be terminated. A single dose of I.V. diltiazem (0.25 mg/kg over 2 minutes) will be administered with 12-lead ECG and BP obtained at 2, 5, 7, 10, 15 and 20 minutes. If at 10 minutes there has been no QT shortening, and the SBP is stable (<20% drop from baseline and > 100 mm Hg) a repeat dose (0.35 mg/kg) will be given at 15 minutes, with 12-lead ECG and BP obtained at 17, 20, 22, 25, 30 and 35 minutes. After 20 minutes (single dose) or 35 minutes (2 doses), the test will end and the subject will remain on continuous telemetry for 2 hours. Prior to discharge the IV will be removed. The primary analysis will be a repeated measures (paired) comparison of the QTc at baseline and following diltiazem.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Age greater than 18 years
- •Genetically confirmed diagnosis of Jervell and Lange-Nielsen syndrome
- •Able to provide written informed consent
排除标准
- •Known hypersensitivity to diltiazem
- •Pregnancy
- •Congestive heart failure, angina, preexcitation, or chronic obstructive pulmonary disease
- •Sick sinus syndrome or atrioventricular block in the absence of a pacemaker/defibrillator
- •Any clinically significant ongoing medical or surgical condition that might jeopardize the subject's safety or interfere with the conduct of the study in the judgement of the investigator
- •Baseline systolic blood pressure less than 100 Hg or diastolic blood pressure less than 60 Hg
研究组 & 干预措施
Diltiazem
IV diltiazem (0.25 mg/kg) will be given over 2 minutes. A second IV dose (0.35 mg/kg) may be given at 10 minutes.
干预措施: diltiazem (Drug)
结局指标
主要结局
QT Interval
时间窗: 5 minutes
QT interval (on 12-lead ECG) in ms
次要结局
未报告次要终点
研究者
Prince Joseph Kannankeril
Professor
Vanderbilt University Medical Center