Arrhythmic Right Ventricular CardiomyopathyNCT00083395
Isoproterenol Challenge to Detect Arrhythmogenic Right Ventricular Cardiomyopathy
An observational study of Arrhythmic Right Ventricular Cardiomyopathy, run by National Institutes of Health Clinical Center (CC) at 1 site in United States.
Trial summary
This study will examine the usefulness of a new test called an isoproterenol challenge in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and family members who may have the disease but do not have clear-cut evidence of it. ARVC is a rare condition that runs in families. Read more about this study
- Who is it for?
- Any ageHealthy volunteers can take part
- Conditions the study is for
- Arrhythmic Right Ventricular Cardiomyopathy
- How many people
- 160 participants
- Where
- 1 siteUnited States
- Study dates
- May 2004to Dec 2005
- Phase
- Observational
- Are there results?
- None posted in the registry
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 Any age
- All sexes
- Healthy volunteers accepted
- Child, Adult, Older adult
Who may be able to join
- Patients with ARVC:
- Either gender, aged greater than 5 years.
- The presence of two major criteria; one major and two minor criteria; or four minor criteria from separate diagnostic categories.
- Normal Volunteers:
- Age and gender matched with ARVC patients, age greater than 18 years.
- No known cardiac disease.
Show all 10Show fewer
- Normal EKG, normal echocardiogram, normal Bruce protocol treadmill exercise test.
- Family Members of Patients with ARVC:
- Either gender, aged greater than 5 years.
- A proband with ARVC
Who cannot join
- Patients with ARVC:
- Pregnancy or lactation excludes tests with potential risk, e.g. radiation, isoprenaline, MRI, but does not exclude 12-lead ECG and echocardiogram.
- Coronary artery disease.
- Known infiltrate or congenital heart disease that could mimic the appearances of ARVC.
- Asthma prevents participation in Isoproterenol infusion.
- Any other condition that would prevent participation in the study.
Show all 19Show fewer
- Normal Volunteers:
- Pregnancy or lactation.
- Asthma.
- Coronary artery disease, hypertension, diabetes, hypertrophic cardiomyopathy, or other known cardiomyopathy.
- Known infiltrative or congenital heart disease that could mimic the appearances of ARVC.
- Any Acute or chronic illness.
- Chronic drug therapy.
- Family Members of Patients with ARVC:
- Pregnancy or lactation excludes tests with potential risk, e.g. radiation, isoprenaline, MRI, but does not exclude 12-lead ECG and echocardiogram.
- Coronary artery disease.
- Known infiltrative or congenital heart disease that could mimic the appearances of ARVC.
- Asthma prevents participation in Isoproterenol infusion.
- Any other condition that would prevent participation in the study.
Trial locations and contacts
1 site in 1 country.
United States1 site
National Institutes of Health Clinical Center (CC)
Bethesda, Maryland, 20892
About this study
Official title: Utility of Isoproterenol Challenge Test to Detect Disease in Patients With Incomplete Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy
This study will examine the usefulness of a new test called an isoproterenol challenge in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and family members who may have the disease but do not have clear-cut evidence of it. ARVC is a rare condition that runs in families. Heart muscle is replaced with fatty, scar-like tissue, especially in the right ventricle (lower pumping chamber of the heart), and can sometimes extend to the left ventricle (the main pumping chamber). The fat can interfere with the heartbeat, producing abnormal heart rhythms, such as ventricular tachycardia (VT) - a very fast heartbeat that can cause sudden death, especially in young people. Isoproterenol is a drug that increases heart rate and heart muscle contractions. In isoproterenol challenge, subjects are given increasing doses of the drug through a catheter (see details below) to try to produce an abnormal heart rhythm.
ARVC is hard to diagnose with current tests. This study will see if isoproterenol challenge provokes VT in patients with the disease and can confirm the diagnosis; if it can detect the disease in family members better than currently available tests; and if it provokes abnormal rhythms in healthy control subjects. In addition, the study will explore the genetics of ARVC and determine whether infection could contribute to its development.
Read more
Patients with ARVC, their family members, and normal volunteers 18 years of age and older may be eligible for this study. Candidates are screened with a medical history and physical examination, electrocardiogram (EKG), treadmill and bicycle exercise testing, and an echocardiogram (ultrasound test of the heart).
Participants undergo the following tests and procedures:
- Blood tests - Blood is collected to study the genetics of ARVC, to test for evidence of old infections, and to measure brain natriuretic peptide - a hormone that can increase with development of heart failure.
- Heart magnetic resonance imaging (MRI). This test looks at heart structure and function. MRI uses a magnetic field and radio waves to produce images of body tissues and organs. The subject lies on a table that is moved into the scanner (a narrow cylinder), wearing earplugs to muffle loud knocking sounds that occur during the scanning process. At some time during the test, the subject is given a contrast agent called gadolinium through a catheter (thin, flexible tube) in a vein to improve the scan pictures. The scan time varies from 30 to 90 minutes, with most scans lasting 60 minutes. (Control subjects do not undergo MRI.)
- Isoproterenol challenge. Subjects are given increasing doses of isoproterenol through a catheter until the heart rate reaches 100 to 120 beats per minute for no more than 1 hour. A special EKG records heart rhythm during the test and an echocardiogram records right and left ventricular function.
- QRST surface mapping EKG. This special EKG, done with 64 or 120 leads, maps abnormalities of heart rhythm and cardiac conduction during the isoproterenol challenge. These tests are like a regular EKG, except that more leads are placed on the chest, and on the back as well.
Patients and family members who wish to have follow-up visits may return to the NIH Clinical Center once a year for 5 years for guidance about therapy based on clinical considerations and new information or investigations.
Read the detailed description
Arrhythmogenic right ventricular cardiomyopathy (ARVC) formerly referred to as arrhythmogenic right ventricular dysplasia (ARVD) is a familial hetergenous clinical and molecular disease characterized by dilatation and dysfunction of the right ventricle and ventricular arrhythmias. The ventricular arrhythmias are heart rate and catecholamine dependent. Not infrequently, there is involvement of the left ventricle. The diagnosis of ARVC is critical as therapy including implantable defibrillators may prevent sudden death. However, identification of affected family members remains a major challenge due to limitation of current imaging and diagnostic techniques. We propose (1) to establish the sensitivity and specificity of the isoproterenol challenge test for ARVC by testing both patients with known ARVC and healthy volunteers; (2) to estimate the proportion of family members who present with incomplete criteria for ARVC but are subsequently diagnosed with the condition by an isoproterenol challenge test; and, (3) to study the inheritance of ARVC and the potential role of occult infection in its development.
- Keywords
- ARVC/ARVD, Genetics, Sudden Death, Heart, MRI, Cardiomyopathy, Arrhythmogenic Right Ventricular, ARVC, Healthy Volunteer, HV
- Medical subject headings
- Death, Sudden, Cardiomyopathies
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted24 May 2004 (estimated)
- Study startMay 2004
- Study completionDec 2005
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (3)Papers the sponsor has linked to this study.
- Other trials for Arrhythmic Right Ventricular CardiomyopathyStudies of the same condition, open and under way.
- 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
- Observational
- Enrolment
- 160 participants
Sponsor and investigators
- Lead sponsor
- National Institutes of Health Clinical Center (CC)
- Sponsor type
- US National Institutes of Health
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT00083395
- Sponsor's protocol ID
- 040194
- Other IDs
- 04-CC-0194
- Status last verified
- Dec 2005
- First submitted
- 22 May 2004
- Last updated
- 4 Mar 2008
- Results posted
- No
Publications
DALLA VOLTA S, BATTAGLIA G, ZERBINI E. "Auricularization' of right ventricular pressure curve. Am Heart J. 1961 Jan;61:25-33. doi: 10.1016/0002-8703(61)90513-0. No abstract available.
PubMed 13719440Basso C, Thiene G, Nava A, Dalla Volta S. Arrhythmogenic right ventricular cardiomyopathy: a survey of the investigations at the University of Padua. Clin Cardiol. 1997 Apr;20(4):333-6. doi: 10.1002/clc.4960200406.
PubMed 9098590Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, Grosgogeat Y. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982 Feb;65(2):384-98. doi: 10.1161/01.cir.65.2.384.
PubMed 7053899
Study at a glance
From the registry record, last updated by the sponsor on 4 Mar 2008. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Utility of Isoproterenol Challenge Test to Detect Disease in Patients With Incomplete Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy
- ClinicalTrials.gov ID
- NCT00083395
- Acronym / sponsor ID
- 040194
- Keywords
- ARVC/ARVD, Genetics, Sudden Death, Heart, MRI, Cardiomyopathy, Arrhythmogenic Right Ventricular, ARVC
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 160 participants
- Lead sponsor
- National Institutes of Health Clinical Center (CC)US National Institutes of Health
- Study start
- May 2004
- Study completion
- Dec 2005
- First posted
- 24 May 2004 (estimated)
- Results
- Not posted in the source record
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 examine the usefulness of a new test called an isoproterenol challenge in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and family members who may have the disease but do not have clear-cut evidence of it. ARVC is a rare condition that runs in families. Heart muscle is replaced with fatty, scar-like tissue, especially in the right ventricle (lower pumping chamber of the heart), and can sometimes extend to the left ventricle (the main pumping chamber). The fat can interfere with the heartbeat, producing abnormal heart rhythms, such as ventricular tachycardia (VT) - a very fast heartbeat that can cause sudden death, especially in young people. Isoproterenol is a drug that increases heart rate and heart muscle contractions. In isoproterenol challenge, subjects are given increasing doses of the drug through a catheter (see details below) to try to produce an abnormal heart rhythm.
ARVC is hard to diagnose with current tests. This study will see if isoproterenol challenge provokes VT in patients with the disease and can confirm the diagnosis; if it can detect the disease in family members better than currently available tests; and if it provokes abnormal rhythms in healthy control subjects. In addition, the study will explore the genetics of ARVC and determine whether infection could contribute to its development.
Patients with ARVC, their family members, and normal volunteers 18 years of age and older may be eligible for this study. Candidates are screened with a medical history and physical examination, electrocardiogram (EKG), treadmill and bicycle exercise testing, and an echocardiogram (ultrasound test of the heart).
Participants undergo the following tests and procedures:
- Blood tests - Blood is collected to study the genetics of ARVC, to test for evidence of old infections, and to measure brain natriuretic peptide - a hormone that can increase with development of heart failure.
- Heart magnetic resonance imaging (MRI). This test looks at heart structure and function. MRI uses a magnetic field and radio waves to produce images of body tissues and organs. The subject lies on a table that is moved into the scanner (a narrow cylinder), wearing earplugs to muffle loud knocking sounds that occur during the scanning process. At some time during the test, the subject is given a contrast agent called gadolinium through a catheter (thin, flexible tube) in a vein to improve the scan pictures. The scan time varies from 30 to 90 minutes, with most scans lasting 60 minutes. (Control subjects do not undergo MRI.)
- Isoproterenol challenge. Subjects are given increasing doses of isoproterenol through a catheter until the heart rate reaches 100 to 120 beats per minute for no more than 1 hour. A special EKG records heart rhythm during the test and an echocardiogram records right and left ventricular function.
- QRST surface mapping EKG. This special EKG, done with 64 or 120 leads, maps abnormalities of heart rhythm and cardiac conduction during the isoproterenol challenge. These tests are like a regular EKG, except that more leads are placed on the chest, and on the back as well.
Patients and family members who wish to have follow-up visits may return to the NIH Clinical Center once a year for 5 years for guidance about therapy based on clinical considerations and new information or investigations.
Detailed description
Arrhythmogenic right ventricular cardiomyopathy (ARVC) formerly referred to as arrhythmogenic right ventricular dysplasia (ARVD) is a familial hetergenous clinical and molecular disease characterized by dilatation and dysfunction of the right ventricle and ventricular arrhythmias. The ventricular arrhythmias are heart rate and catecholamine dependent. Not infrequently, there is involvement of the left ventricle. The diagnosis of ARVC is critical as therapy including implantable defibrillators may prevent sudden death. However, identification of affected family members remains a major challenge due to limitation of current imaging and diagnostic techniques. We propose (1) to establish the sensitivity and specificity of the isoproterenol challenge test for ARVC by testing both patients with known ARVC and healthy volunteers; (2) to estimate the proportion of family members who present with incomplete criteria for ARVC but are subsequently diagnosed with the condition by an isoproterenol challenge test; and, (3) to study the inheritance of ARVC and the potential role of occult infection in its development.
Study design
Who may qualify
- Sex
- All
- Healthy volunteers
- Accepted
- Age groups
- Child, Adult, Older adult
Key inclusion criteria (10 listed)
- Either gender, aged greater than 5 years.
- The presence of two major criteria; one major and two minor criteria; or four minor criteria from separate diagnostic categories.
- Age and gender matched with ARVC patients, age greater than 18 years.
- No known cardiac disease.
- Normal EKG, normal echocardiogram, normal Bruce protocol treadmill exercise test.
Key exclusion criteria (19 listed)
- Pregnancy or lactation excludes tests with potential risk, e.g. radiation, isoprenaline, MRI, but does not exclude 12-lead ECG and echocardiogram.
- Coronary artery disease.
- Known infiltrate or congenital heart disease that could mimic the appearances of ARVC.
- Asthma prevents participation in Isoproterenol infusion.
- Any other condition that would prevent participation in the study.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Not reported in the source record.
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 4 Mar 2008.
- Background: PubMed 13719440DALLA VOLTA S, BATTAGLIA G, ZERBINI E. "Auricularization' of right ventricular pressure curve. Am Heart J. 1961 Jan;61:25-33. doi: 10.1016/0002-8703(61)90513-0. No abstract available.
- Background: PubMed 9098590Basso C, Thiene G, Nava A, Dalla Volta S. Arrhythmogenic right ventricular cardiomyopathy: a survey of the investigations at the University of Padua. Clin Cardiol. 1997 Apr;20(4):333-6. doi: 10.1002/clc.4960200406.
- Background: PubMed 7053899Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, Grosgogeat Y. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982 Feb;65(2):384-98. doi: 10.1161/01.cir.65.2.384.
Related trials for Arrhythmic Right Ventricular Cardiomyopathy
Not yet recruitingObservational study
Treatment Registry of Arrhythmias, Complications and Electrocardiograms in Arrhythmogenic CardioMyopathies
Arrhythmogenic Cardiomyopathy (AC, ARVD/C), Ventricular Tachycardia (VT)
RecruitingObservational study
Distribution of Cell-cell Junction Proteins in Arrhythmic Disorders
Arrhythmogenic Right Ventricular Dysplasia, Brugada Syndrome
Active, not recruiting
Catheter Ablation Versus Anti-arrhythmic Drugs for Ventricular Tachycardia
Heart Disease Structural Disorder, Ventricular Tachycardia
RecruitingObservational study
Multimodal and Multidisciplinary Approach to Optimize Diagnostic, Prognostic, and Therapeutic Management of Patients with Non-ischemic Cardiomyopathies and Arrhythmogenic-inflammatory Phenotypes: a Multicenter, Observational, Retrospective and Prospective Registry Study.
Non-ischemic Cardiomyopathy, Dilated Cardiomyopathy (DCM)
Enrolling by invitationObservational study
Hearts in Rhythm Organization (HiRO)National Registry and Bio Bank
Sudden Cardiac Arrest, Sudden Arrhythmic Death 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.
The record lists no contact. Use the official record to reach the sponsor.
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Arrhythmic Right Ventricular Cardiomyopathy, run by National Institutes of Health Clinical Center (CC) 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 10 inclusion and 19 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 4 Mar 2008. 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.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
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.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT00083395, which also lists 3 linked publications.
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 4 Mar 2008.