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Sponsored by: |
National Institutes of Health Clinical Center (CC) |
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Information provided by: | National Institutes of Health Clinical Center (CC) |
ClinicalTrials.gov Identifier: | NCT00083395 |
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:
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.
Condition |
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Arrhythmic Right Ventricular Cardiomyopathy |
Study Type: | Observational |
Official Title: | Utility of Isoproterenol Challenge Test to Detect Disease in Patients With Incomplete Diagnostic Criteria for Arrhythmogenic Right Ventricular Cardiomyopathy |
Estimated Enrollment: | 160 |
Study Start Date: | May 2004 |
Estimated Study Completion Date: | December 2005 |
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.
Genders Eligible for Study: | Both |
Accepts Healthy Volunteers: | Yes |
INCLUSION CRITERIA:
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.
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
EXCLUSION CRITERIA:
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.
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.
Study ID Numbers: | 040194, 04-CC-0194 |
Study First Received: | May 22, 2004 |
Last Updated: | March 3, 2008 |
ClinicalTrials.gov Identifier: | NCT00083395 History of Changes |
Health Authority: | United States: Federal Government |
ARVC/ARVD Genetics Sudden Death Heart MRI Cardiomyopathy |
Arrhythmogenic Right Ventricular ARVC Sudden Death Healthy Volunteer HV |
Neurotransmitter Agents Death Heart Diseases Cardiovascular Abnormalities Adrenergic Agents Adrenergic beta-Agonists Anti-Asthmatic Agents Cardiovascular Agents Healthy |
Cardiomyopathies Isoproterenol Adrenergic Agonists Death, Sudden Peripheral Nervous System Agents Congenital Abnormalities Bronchodilator Agents Heart Defects, Congenital Arrhythmogenic Right Ventricular Dysplasia |
Respiratory System Agents Neurotransmitter Agents Molecular Mechanisms of Pharmacological Action Adrenergic Agents Cardiotonic Agents Physiological Effects of Drugs Adrenergic Agonists Therapeutic Uses Cardiovascular Diseases Congenital Abnormalities Heart Diseases Adrenergic beta-Agonists Cardiovascular Abnormalities |
Sympathomimetics Anti-Asthmatic Agents Cardiovascular Agents Cardiomyopathies Protective Agents Isoproterenol Pharmacologic Actions Autonomic Agents Peripheral Nervous System Agents Heart Defects, Congenital Bronchodilator Agents Arrhythmogenic Right Ventricular Dysplasia |