NCT00011648

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.

Trial Health

80
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
986

participants targeted

Target at P75+ for all trials

Geographic Reach
1 country

2 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

February 24, 2001

Completed
2 days until next milestone

First Posted

Study publicly available on registry

February 26, 2001

Completed
7 years until next milestone

Study Start

First participant enrolled

February 19, 2008

Completed
Last Updated

April 20, 2026

Status Verified

February 17, 2026

First QC Date

February 24, 2001

Last Update Submit

April 17, 2026

Conditions

Sickle Cell AnemiaSickle Cell DiseasePulmonary Hypertension

Keywords

EchocardiogramMorbidityMortalityNatural HistorySickle Cell AnemiaSecondary Pulmonary Hypertension

Outcome Measures

Primary Outcomes (1)

  • To determine the prevalence and prognosis of secondary pulmonary hypertension in adult patients with sickle cell anemia.

    predictive of any clinical outcome or response in sickle cell disease will provide preliminary evidence for further investigation

    10 years

Secondary Outcomes (1)

  • To determine whether genetic polymorphisms in candidate genes contribute to its development or response to treatment.

    1 year

Study Arms (2)

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

Eligibility Criteria

Age18 Years+
Sexall
Healthy VolunteersNo
Age GroupsAdult (18-64), Older Adult (65+)
Sampling MethodNon-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)

You may qualify if:

  • 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).

You may not qualify if:

  • Hb A-only phenotype and sickle cell trait.
  • Decisionally impaired subjects.
  • Pregnant or lactating women
  • Male and females African American subjects over 18 years of age.
  • Diagnosis of sickle cell disease (electrophoretic documentation of SS, or SC, or SB thallassemia genotype is required.)
  • Decisionally impaired subjects.
  • Pregnant or lactating women

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (2)

Howard University Hospital

Washington D.C., District of Columbia, 20060, United States

Location

National Institutes of Health Clinical Center

Bethesda, Maryland, 20892, United States

Location

Related Publications (12)

  • Li H, Gao S, Wang X, Asomaning N, Conrey A, Kruah B, Joo J, Wu CO, Sachdev V, Thein SL. Transthyretin V122I Variant and Protein Affects Cardiac Severity and Mortality in Sickle Cell Disease. Blood Adv. 2026 Apr 1:bloodadvances.2026019698. doi: 10.1182/bloodadvances.2026019698. Online ahead of print.

    PMID: 41921073DERIVED

  • Parrow NL, Doherty JM, Conrey A, Thein SL, Fleming RE. Relationships Between Markers of Iron Status and Hematological Parameters in Patients With Sickle Cell Disease. Adv Hematol. 2024 Dec 3;2024:9872440. doi: 10.1155/ah/9872440. eCollection 2024.

    PMID: 39659429DERIVED

  • van Vuren AJ, Minniti CP, Mendelsohn L, Baird JH, Kato GJ, van Beers EJ. Lactate dehydrogenase to carboxyhemoglobin ratio as a biomarker of heme release to heme processing is associated with higher tricuspid regurgitant jet velocity and early death in sickle cell disease. Am J Hematol. 2021 Sep 1;96(9):E315-E318. doi: 10.1002/ajh.26243. Epub 2021 Jun 10. No abstract available.

    PMID: 34000072DERIVED

  • Sachdev V, Tian X, Gu Y, Nichols J, Sidenko S, Li W, Beri A, Layne WA, Allen D, Wu CO, Thein SL. A phenotypic risk score for predicting mortality in sickle cell disease. Br J Haematol. 2021 Mar;192(5):932-941. doi: 10.1111/bjh.17342. Epub 2021 Jan 28.

    PMID: 33506990DERIVED

  • Nguyen KL, Tian X, Alam S, Mehari A, Leung SW, Seamon C, Allen D, Minniti CP, Sachdev V, Arai AE, Kato GJ. Elevated transpulmonary gradient and cardiac magnetic resonance-derived right ventricular remodeling predict poor outcomes in sickle cell disease. Haematologica. 2016 Feb;101(2):e40-3. doi: 10.3324/haematol.2015.125229. Epub 2015 Nov 20. No abstract available.

    PMID: 26589907DERIVED

  • Fitzhugh CD, Hsieh MM, Allen D, Coles WA, Seamon C, Ring M, Zhao X, Minniti CP, Rodgers GP, Schechter AN, Tisdale JF, Taylor JG 6th. Hydroxyurea-Increased Fetal Hemoglobin Is Associated with Less Organ Damage and Longer Survival in Adults with Sickle Cell Anemia. PLoS One. 2015 Nov 17;10(11):e0141706. doi: 10.1371/journal.pone.0141706. eCollection 2015.

    PMID: 26576059DERIVED

  • Wallen GR, Minniti CP, Krumlauf M, Eckes E, Allen D, Oguhebe A, Seamon C, Darbari DS, Hildesheim M, Yang L, Schulden JD, Kato GJ, Taylor JG 6th. Sleep disturbance, depression and pain in adults with sickle cell disease. BMC Psychiatry. 2014 Jul 21;14:207. doi: 10.1186/1471-244X-14-207.

    PMID: 25047658DERIVED

  • Darbari DS, Wang Z, Kwak M, Hildesheim M, Nichols J, Allen D, Seamon C, Peters-Lawrence M, Conrey A, Hall MK, Kato GJ, Taylor JG 6th. Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. PLoS One. 2013 Nov 5;8(11):e79923. doi: 10.1371/journal.pone.0079923. eCollection 2013.

    PMID: 24224021DERIVED

  • Nekhai S, Xu M, Foster A, Kasvosve I, Diaz S, Machado RF, Castro OL, Kato GJ, Taylor JG 6th, Gordeuk VR. Reduced sensitivity of the ferroportin Q248H mutant to physiological concentrations of hepcidin. Haematologica. 2013 Mar;98(3):455-63. doi: 10.3324/haematol.2012.066530. Epub 2012 Oct 12.

    PMID: 23065513DERIVED

  • Minniti CP, Taylor JG 6th, Hildesheim M, O'Neal P, Wilson J, Castro O, Gordeuk VR, Kato GJ. Laboratory and echocardiography markers in sickle cell patients with leg ulcers. Am J Hematol. 2011 Aug;86(8):705-8. doi: 10.1002/ajh.22065. Epub 2011 May 31.

    PMID: 21630312DERIVED

  • Sundaram N, Tailor A, Mendelsohn L, Wansapura J, Wang X, Higashimoto T, Pauciulo MW, Gottliebson W, Kalra VK, Nichols WC, Kato GJ, Malik P. High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension. Blood. 2010 Jul 8;116(1):109-12. doi: 10.1182/blood-2009-09-244830. Epub 2010 Mar 24.

    PMID: 20335221DERIVED

  • Kato GJ, Wang Z, Machado RF, Blackwelder WC, Taylor JG 6th, Hazen SL. Endogenous nitric oxide synthase inhibitors in sickle cell disease: abnormal levels and correlations with pulmonary hypertension, desaturation, haemolysis, organ dysfunction and death. Br J Haematol. 2009 May;145(4):506-13. doi: 10.1111/j.1365-2141.2009.07658.x. Epub 2008 Mar 17.

    PMID: 19344390DERIVED

Related Links

MeSH Terms

Conditions

Hypertension, PulmonaryAnemia, Sickle Cell

Condition Hierarchy (Ancestors)

Lung DiseasesRespiratory Tract DiseasesHypertensionVascular DiseasesCardiovascular DiseasesAnemia, Hemolytic, CongenitalAnemia, HemolyticAnemiaHematologic DiseasesHemic and Lymphatic DiseasesHemoglobinopathiesGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

  • Swee Lay Thein, M.D.

    National Heart, Lung, and Blood Institute (NHLBI)

    PRINCIPAL INVESTIGATOR

Study Design

Study Type
observational
Observational Model
CASE CONTROL
Time Perspective
PROSPECTIVE
Sponsor Type
NIH
Responsible Party
SPONSOR

Study Record Dates

First Submitted

February 24, 2001

First Posted

February 26, 2001

Study Start

February 19, 2008

Last Updated

April 20, 2026

Record last verified: 2026-02-17

Locations

US(2)