Ignite Creation Date:
2024-05-05 @ 11:42 AM
Last Modification Date:
2024-10-26 @ 9:12 AM
Study NCT ID:
NCT00113152
Status:
COMPLETED
Last Update Posted:
2017-07-02
First Post:
2005-06-03
Brief Title:
Heart Disease in Sickle Cell Anemia
Sponsor:
National Institutes of Health Clinical Center CC
Organization:
National Institutes of Health Clinical Center CC
Study Overview
Official Title:
Are Left Ventricular Abnormalities Associated With Pulmonary Hypertension in Sickle Cell Anemia
Status:
COMPLETED
Status Verified Date:
2007-10-02
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
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
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
Detailed Description:
Sickle cell anemia is an inherited blood disorder primarily affecting groups with origins in endemic malarial areas especially those of African descent SCA results from one of two single amino-acid substitutions in beta-hemoglobin Hb-S and Hb-C that increases the propensity for hemoglobin to polymerize thus distorting sickling and hemolyzing red cells Individuals homozygous for Hb-S or double heterozygote Hb-S and Hb-C develop sickle cell anemia SCA while heterozygotes have sickle cell trait SCA is characterized by chronic anemia and crises of red cell sickling and ischemia that are often painful and affect several organs and tissue types SCA confers considerable disability morbidity and mortality
Annual mortality from SCA has been estimated at approximately 3 As a significant number of these deaths are sudden a cardiac cause has been suspected However no cardiac mechanism of sudden death SD has been clearly identified Recently it has been demonstrated that SCA patients with pulmonary hypertension PAH have a higher incidence of SD than those with normal pulmonary pressures In many patients PAH occurs in association with elevated pulmonary arterial wedge pressures and normal pulmonary arterial resistance suggesting that the PAH develops as the result of left ventricular LV abnormalities Furthermore in other conditions in which PAH develops SD occurs only at pressures considerably higher than those observed in SCA These factors suggest that PAH in SCA is a surrogate marker for rather than the cause of SD Rather an SCA cardiomyopathic process may provide a unifying mechanism that associates moderate degrees of PAH and high risk of SD from cardiac causes
We propose to describe the extent of cardiac involvement in SCA Specifically we will 1 describe the LV volume-pressure relations in SCA patients with and without pulmonary hypertension in order to determine how elevated pulmonary pressures are related to dynamic filling properties of the LV and 2 determine whether cardiac arrhythmias are common in SCA patients with PAH and if they contribute to SD
Improved understanding of the etiology and mechanisms of SD in SCA may allow the development and testing of therapies for the primary prevention of SD
Annual mortality from SCA has been estimated at approximately 3 As a significant number of these deaths are sudden a cardiac cause has been suspected However no cardiac mechanism of sudden death SD has been clearly identified Recently it has been demonstrated that SCA patients with pulmonary hypertension PAH have a higher incidence of SD than those with normal pulmonary pressures In many patients PAH occurs in association with elevated pulmonary arterial wedge pressures and normal pulmonary arterial resistance suggesting that the PAH develops as the result of left ventricular LV abnormalities Furthermore in other conditions in which PAH develops SD occurs only at pressures considerably higher than those observed in SCA These factors suggest that PAH in SCA is a surrogate marker for rather than the cause of SD Rather an SCA cardiomyopathic process may provide a unifying mechanism that associates moderate degrees of PAH and high risk of SD from cardiac causes
We propose to describe the extent of cardiac involvement in SCA Specifically we will 1 describe the LV volume-pressure relations in SCA patients with and without pulmonary hypertension in order to determine how elevated pulmonary pressures are related to dynamic filling properties of the LV and 2 determine whether cardiac arrhythmias are common in SCA patients with PAH and if they contribute to SD
Improved understanding of the etiology and mechanisms of SD in SCA may allow the development and testing of therapies for the primary prevention of SD
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
None
Is a FDA Regulated Device?:
None
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None
Secondary IDs
| Secondary ID | Type | Domain | Link |
|---|---|---|---|
| 05-CC-0154 | None | None | None |