Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia

Sponsor
National Heart, Lung, and Blood Institute (NHLBI) (NIH)
Overall Status
Completed
CT.gov ID
NCT00011648
Collaborator
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) (NIH)
986
1

Study Details

Study Description

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 Intervention/Treatment Phase

    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

    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 :
    Feb 19, 2008

    Arms and Interventions

    Arm Intervention/Treatment
    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

    Primary Outcome Measures

    1. To determine the prevalence and prognosis of secondary pulmonary hypertension in adult patients with sickle cell anemia. [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. [1 year]

    Eligibility Criteria

    Criteria

    Ages Eligible for Study:
    18 Years and Older
    Sexes Eligible for Study:
    All
    Accepts Healthy Volunteers:
    No
    • 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

    Locations

    Site City State Country Postal Code
    1 National Institutes of Health Clinical Center, 9000 Rockville Pike 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

    • Principal Investigator: Swee Lay Thein, M.D., National Heart, Lung, and Blood Institute (NHLBI)

    Study Documents (Full-Text)

    None provided.

    More Information

    Additional Information:

    Publications

    None provided.
    Responsible Party:
    National Heart, Lung, and Blood Institute (NHLBI)
    ClinicalTrials.gov Identifier:
    NCT00011648
    Other Study ID Numbers:
    • 010088
    • 01-H-0088
    First Posted:
    Feb 26, 2001
    Last Update Posted:
    Aug 25, 2022
    Last Verified:
    Aug 4, 2022
    Studies a U.S. FDA-regulated Drug Product:
    No
    Studies a U.S. FDA-regulated Device Product:
    No
    Keywords provided by National Heart, Lung, and Blood Institute (NHLBI)
    Additional relevant MeSH terms:

    Study Results

    No Results Posted as of Aug 25, 2022