Jervell and Lange Nielsen SyndromePhase 4NCT06534671
Diltiazem in Jervell and Lange-Nielsen Syndrome
A Phase 4 study testing diltiazem in people with Jervell and Lange Nielsen Syndrome, run by Vanderbilt University Medical Center at 1 site in United States.
Trial summary
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. Read more about this study
- Who is it for?
- Ages 18 years and older
- Conditions the study is for
- Jervell and Lange Nielsen Syndrome
- What is being tested?
- diltiazem
- Type of treatment
- Injection or infusion
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 1 participants
- Where
- 1 siteUnited States
- Study dates
- 16 Oct 2024to 23 Oct 2024
- Phase
- Phase 4
- Are there results?
- Yes, posted in the registrySummary in the doctors' view
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 years and older
- All sexes
- No healthy volunteers
- Adult, Older adult
Who may be able to join
- Age greater than 18 years
- Genetically confirmed diagnosis of Jervell and Lange-Nielsen syndrome
- Able to provide written informed consent
Who cannot join
- 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
Trial locations and contacts
1 site in 1 country.
United States1 site
Vanderbilt University Medical Center
Nashville, Tennessee, 37232
About this study
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.
Read the detailed description
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.
- Conditions
- Jervell and Lange Nielsen Syndrome
- Being tested
- diltiazem
- Keywords
- Long QT syndrome
- Medical subject headings
- Jervell-Lange Nielsen Syndrome, Long QT Syndrome
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
diltiazemDrug
IV diltiazem (0.25 mg/kg) will be given over 2 minutes, with ECG and blood pressure measurements at baseline, 2, 5, 7, 10, 15 and 20 minutes. If at 10 minutes there is no QT shortening, and blood pressure is stable, an additional dose (0.35 mg/kg) will be given with ECG and blood pressure measurements at 17, 20, 22, 25, 30 and 35 minutes.
| Study group | Role | What they receive |
|---|---|---|
| Diltiazem | Experimental | 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. Receives: Drug: diltiazem |
What is being measured
The primary outcome is the main question the study is designed to answer.
QT Interval
QT interval (on 12-lead ECG) in ms
Measured: Baseline
QT Interval
QT interval (on 12-lead ECG) in ms
Measured: 5 minutes
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted2 Aug 2024
- Study start16 Oct 2024
- Primary completion23 Oct 2024
- Study completion23 Oct 2024
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Posted resultsParticipant flow, outcome measures and adverse events reported by the sponsor.
- Other trials for Jervell and Lange Nielsen SyndromeStudies of the same condition, open and under way.
- More trials run by Vanderbilt University Medical CenterThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase 4
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Enrolment
- 1 participants (actual)
Sponsor and investigators
- Lead sponsor
- Vanderbilt University Medical Center
- Sponsor type
- University, hospital or other
- Responsible party
- Principal investigator, Prince Joseph Kannankeril, Vanderbilt University Medical Center
- Investigators
- Prince J Kannankeril, MD, MSCI, Principal investigator, Vanderbilt University Medical Center
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT06534671
- Sponsor's protocol ID
- 240535
- Status last verified
- Sep 2025
- First submitted
- 29 Jul 2024
- Last updated
- 14 Oct 2025
- Results posted
- Yes, on ClinicalTrials.gov
- Data monitoring committee
- No
- FDA-regulated drug
- Yes
- FDA-regulated device
- No
- Individual data sharing
- Yes: Individual-level QT results will be published. Data obtained through this study may be provided to qualified researchers with no PHI included.
Study at a glance
From the registry record, last updated by the sponsor on 14 Oct 2025. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Diltiazem in Jervell and Lange-Nielsen Syndrome
- ClinicalTrials.gov ID
- NCT06534671
- Acronym / sponsor ID
- 240535
- Conditions
- Jervell and Lange Nielsen Syndrome
- Keywords
- Long QT syndrome
- Phase
- Phase 4
- Study type
- Interventional
- Recruitment status
- Completed
- Enrolment
- 1 participants (actual)
- Lead sponsor
- Vanderbilt University Medical CenterUniversity, hospital or other
- Study start
- 16 Oct 2024 (actual)
- Primary completion
- 23 Oct 2024 (actual)
- Study completion
- 23 Oct 2024 (actual)
- First posted
- 2 Aug 2024 (actual)
- Results
- Posted 14 Oct 2025
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Detailed description
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.
Study design
- Primary purpose
- Treatment
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Diltiazem | Experimental | Drug: diltiazemIV 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. |
Who may qualify
- Ages
- 18 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
Key inclusion criteria (3 listed)
- Age greater than 18 years
- Genetically confirmed diagnosis of Jervell and Lange-Nielsen syndrome
- Able to provide written informed consent
Key exclusion criteria (6 listed)
- 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
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| diltiazemIV diltiazem (0.25 mg/kg) will be given over 2 minutes, with ECG and blood pressure measurements at baseline, 2, 5, 7, 10, 15 and 20 minutes. If at 10 minutes there is no QT shortening, and blood pressure is stable, an additional dose (0.35 mg/kg) will be given with ECG and blood pressure measurements at 17, 20, 22, 25, 30 and 35 minutes. | Drug | Diltiazem |
Primary outcome
QT Interval
Time frame: Baseline
QT interval (on 12-lead ECG) in ms
QT Interval
Time frame: 5 minutes
QT interval (on 12-lead ECG) in ms
Results and safety
Loading the posted results from ClinicalTrials.gov…
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 14 Oct 2025.
- Posted resultsParticipant flow, baseline, all outcome measures and adverse events.
- Individual participant dataSharing planned: Yes. Individual-level QT results will be published. Data obtained through this study may be provided to qualified researchers with no PHI included.
Related trials for Jervell and Lange Nielsen Syndrome
Active, not recruitingObservational study
Early Check: Expanded Screening in Newborns
Spinal Muscular Atrophy, Fragile X Syndrome
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Prince J Kannankeril, MD, MSCI principal investigator
Vanderbilt University Medical Center
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A Phase 4 study testing diltiazem in people with Jervell and Lange Nielsen Syndrome, run by Vanderbilt University Medical Center at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 3 inclusion and 6 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 14 Oct 2025. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
What does this phase mean?
Phase 4 studies take place after a treatment is approved, to learn more about its long-term benefits and risks in everyday use.
Not every study follows the same path, and some studies (observational studies, device studies) have no phase.
Are results available for this study?
Yes. Results are posted in the registry (first posted 14 Oct 2025). A summary is in the For doctors view, and the full results are on ClinicalTrials.gov.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
QT Interval (time frame: Baseline). The study lists 2 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 14 Oct 2025.