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Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our disclaimer for details.
 
ClinicalTrials.gov Identifier: NCT00011648
Recruitment Status : Completed
First Posted : February 26, 2001
Last Update Posted : May 25, 2023
Sponsor:
Collaborator:
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Information provided by (Responsible Party):
National Institutes of Health Clinical Center (CC) ( National Heart, Lung, and Blood Institute (NHLBI) )

Study Description
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Brief Summary:

The purpose of this study is to determine how often people with sickle cell anemia develop pulmonary hypertension a serious disease in which blood pressure in the artery to the lungs is elevated.

Men and women 18 years of age and older with sickle cell anemia may be eligible for this study. Participants will undergo an evaluation at Howard University s Comprehensive Sickle Cell Center in Washington, D.C. or at the National Institutes of Health in Bethesda, Maryland. It will include the following:

  • medical history
  • physical examination
  • blood collection (no more than 50 ml., or about 1/3 cup) to confirm the diagnosis of sickle cell anemia, sickle cell trait or beta-thalassemia (Some blood will be stored for future research testing on sickle cell anemia.)
  • echocardiogram (ultrasound test of the heart) to check the pumping action of the heart and the rate at which blood travels through the tricuspid valve.

Following this evaluation, a study nurse will contact participants twice a month for 2 months and then once every 3 months for the next 3 years for a telephone interview. The interview will include questions about general health and recent health-related events, such as hospitalizations or emergency room visits.


Condition or disease
Pulmonary Hypertension Sickle Cell Anemia Sickle Cell Disease

Detailed Description:

Sickle cell anemia is an autosomal recessive disorder and the most common genetic disease affecting African-Americans. Approximately 0.15% of African-Americans are homozygous for sickle cell disease, and 8% have sickle cell trait. Acute pain crisis, acute chest syndrome (ACS), and secondary pulmonary hypertension are common complications of sickle cell anemia. Mortality rates of sickle cell patients with pulmonary hypertension are significantly increased as compared to patients without pulmonary hypertension. Recent studies report up to 40% mortality at 22 months after detection of elevated pulmonary artery pressures in sickle cell patients. Furthermore, pulmonary hypertension is thought to occur in up to 30% of clinic patients with sickle cell anemia.

This study is designed to determine the prevalence and prognosis of secondary pulmonary hypertension in adult patients with sickle cell anemia, and to determine whether genetic polymorphisms in candidate genes contribute to its development or response to treatment.

Study Design
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Study Type : Observational
Actual Enrollment : 986 participants
Observational Model: Case-Control
Time Perspective: Prospective
Official Title: Determining the Prevalence and Prognosis of Secondary Pulmonary Hypertension in Adult Patients With Sickle Cell Anemia
Actual Study Start Date : February 19, 2008

Resource links provided by the National Library of Medicine


Groups and Cohorts
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Group/Cohort
non-SCD
200 Men and Women without a diagnosis of sickle cell disease 18 years of age or older
SCD
1000 Men and Women with a diagnosis of sickle cell disease


Outcome Measures
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Primary Outcome Measures :
  1. To determine the prevalence and prognosis of secondary pulmonary hypertension in adult patients with sickle cell anemia. [ Time Frame: 10 years ]
    predictive of any clinical outcome or response in sickle cell disease will provide preliminary evidence for further investigation


Secondary Outcome Measures :
  1. To determine whether genetic polymorphisms in candidate genes contribute to its development or response to treatment. [ Time Frame: 1 year ]

Eligibility Criteria
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Information from the National Library of Medicine

Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the contacts provided below. For general information, Learn About Clinical Studies.


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Ages Eligible for Study:   18 Years and older   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Male and females African American subjects over 18 years of age. Exclusion of sickle cell disease (electrophoretic documentation of hemoglobin A is required)
Criteria
  • INCLUSION CRITERIA FOR SICKLE CELL PATIENTS:
  • Male and females over 18 years of age.
  • Diagnosis of sickle cell disease (electrophoretic documentation of SS, SC, or S-beta thallassemia genotype is required).

EXCLUSION CRITERIA FOR SICKLE CELL PATIENTS:

  • Hb A-only phenotype and sickle cell trait.
  • Decisionally impaired subjects.
  • Pregnant or lactating women

INCLUSION CRITERIA FOR CONTROL SUBJECTS:

  • Male and females African American subjects over 18 years of age.
  • Exclusion of sickle cell disease (electrophoretic documentation of hemoglobin A is required).

EXCLUSION CRITERIA FOR CONTROL SUBJECTS:

  • Diagnosis of sickle cell disease (electrophoretic documentation of SS, or SC, or SB thallassemia genotype is required.)
  • Decisionally impaired subjects.
  • Pregnant or lactating women
Contacts and Locations
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Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT00011648


Locations
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United States, Maryland
National Institutes of Health Clinical Center
Bethesda, Maryland, United States, 20892
Sponsors and Collaborators
National Heart, Lung, and Blood Institute (NHLBI)
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Investigators
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Principal Investigator: Swee Lay Thein, M.D. National Heart, Lung, and Blood Institute (NHLBI)
More Information
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Additional Information:

Publications automatically indexed to this study by ClinicalTrials.gov Identifier (NCT Number):

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Responsible Party: National Heart, Lung, and Blood Institute (NHLBI)
ClinicalTrials.gov Identifier: NCT00011648    
Other Study ID Numbers: 010088
01-H-0088
First Posted: February 26, 2001    Key Record Dates
Last Update Posted: May 25, 2023
Last Verified: January 24, 2023

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Keywords provided by National Institutes of Health Clinical Center (CC) ( National Heart, Lung, and Blood Institute (NHLBI) ):
Echocardiogram
Morbidity
Mortality
 Natural History
Sickle Cell Anemia
Secondary Pulmonary Hypertension
Additional relevant MeSH terms:
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Hypertension, Pulmonary
Hypertension
Anemia
Anemia, Sickle Cell
Vascular Diseases
Cardiovascular Diseases
Hematologic Diseases
 Lung Diseases
Respiratory Tract Diseases
Anemia, Hemolytic, Congenital
Anemia, Hemolytic
Hemoglobinopathies
Genetic Diseases, Inborn