Heart Disease in Sickle Cell Anemia

NCT00113152 | Completed

• 2 other identifiers: 05015405-CC-0154
• Study Type: observational
• Target: 120
• Locations: 1 country
• Sites: 2

Brief Summary

This study will explore what may cause people with sickle cell anemia to have heart problems and an increased risk of sudden death. People 18 years of age and older with sickle cell anemia may be eligible for this study. Candidates are screened with a medical history and physical examination, electrocardiogram (EKG), echocardiogram (heart ultrasound), and blood tests. Participants undergo the following tests and procedures:

• Holter monitoring: The patient wears a small, battery-operated device to record heart rate and rhythm over 24 to 48 hours.
• QRST surface mapping: An EKG using 64 electrodes is done at rest and during exercise to provide a detailed look at the heart and its conduction system.
• Chest x-rays are taken to examine the lungs.
• Bicycle exercise echocardiography test: Blood pressure, pulse, heart rhythm and oxygen use are monitored while the patient exercises on a stationary bicycle. Ultrasound pictures are also obtained during the exercise.
• Echocardiogram: A heart ultrasound is done to check how well the heart is pumping blood.
• Pulmonary artery catheterization: A catheter (plastic tube) is inserted into a vein and advanced to the chambers of the heart, through the heart valve and into the lung artery. The pressures in the heart and lung blood vessels are measured while the patient is resting and during exercise, with the bed tilted up and down, and after giving 500 mls of fluid into a vein.
• Blood tests are done to measure a hormone called brain natriuretic peptide, which can increase with the development of heart failure, and nitrite, a substance that can affect blood vessel dilation. Some blood is stored to test for inflammatory markers and for possible future gene and protein analysis.
• Cardiac magnetic resonance imaging (cMRI): The patient lies in a donut-shaped magnet while pictures of the heart are obtained using a magnetic field and radio waves. Earplugs are worn to muffle the loud sounds that occur with electrical switching of the magnetic fields. A contrast agent called gadolinium may be injected to enhance the quality of the images.
• Invasive electrocardiographic (reveal) monitoring: This procedure permits study of the heart rhythms over a long time period. A small device is placed just under the skin on the left side of the chest. It can be left in for up to 14 months to monitor the heartbeat continuously during this time.

Conditions

Sickle Cell Anemia; Pulmonary Hypertension

Keywords

Cardiac Arrhythmias; Diastolic Dysfunction; Pulmonary Hypertension; Sickle Cell Anemia; Sudden Death; Thalassemia

Eligibility Criteria

• Age: 18 Years - 60 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64)

You may qualify if:

• Either gender, aged greater than 18 years
• Diagnosis of sickle cell disease (electrophoretic documentation of SS, SC, or S Beta-thalassemia genotype is required)
• Hematocrit greater than 18 % (with an absolute reticulocyte count greater than 100,000/ml)

You may not qualify if:

• Pregnancy or lactation
• Known or suspected coronary artery disease
• Hematocrit less than 18 %: will not be eligible for the study; may return for evaluation at a later date
• Significant renal insufficiency (patient on hemodialysis or estimated creatinine clearance less than 30% of normal
• Cerebrovascular accident within the last six weeks
• New diagnosis of pulmonary embolism within the last three months
• History of retinal detachment
• Patients with PAH known to be secondary to other causes, such as systemic lupus erythematosus, other collagen vascular diseases, valvular heart disease, congenital heart disease
• Poor echo windows
• Any other condition that would prevent participation in the study (for example HIV infection)
• Either gender, aged greater than 18 years
• Diagnosis of sickle cell disease (electrophoretic documentation of SS, SC, or S Beta-thalassemia genotype is required)
• Pregnancy or lactation
• Known or suspected coronary artery disease
• Significant renal insufficiency (patient on hemodialysis or estimated creatinine clearance less than 30%of normal

Sponsors & Collaborators

• National Institutes of Health Clinical Center (CC): lead

Study Sites (2)

Suburban Hospital

Bethesda, Maryland, 20814, United States

Location

National Institutes of Health Clinical Center, 9000 Rockville Pike

Bethesda, Maryland, 20892, United States

Location

Related Publications (1)

• Charache S. Sickle cells and sudden death. J Lab Clin Med. 1994 Oct;124(4):473-4. No abstract available.

  PMID: 7930871 BACKGROUND

MeSH Terms

Conditions

Anemia, Sickle Cell; Hypertension, Pulmonary; Arrhythmias, Cardiac; Death, Sudden; Thalassemia

Condition Hierarchy (Ancestors)

Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Anemia; Hematologic Diseases; Hemic and Lymphatic Diseases; Hemoglobinopathies; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases; Cardiovascular Diseases; Heart Diseases; Pathologic Processes; Pathological Conditions, Signs and Symptoms; Death

Study Design

• Study Type: observational
• Sponsor Type: NIH

Study Record Dates

• First Submitted: June 3, 2005
• First Posted: June 6, 2005
• Study Start: June 2, 2005
• Study Completion: October 2, 2007
• Last Updated: July 2, 2017

Record last verified: 2007-10-02

Locations

US (2)