Microvessels and Heart Problems in Sickle Cell Disease

NCT01602809 | Completed

• 2 other identifiers: 12012412-H-0124
• Study Type: observational
• Target: 65
• Locations: 1 country
• Sites: 1

Brief Summary

Background: \- Small blood vessels (microvessels) in many different organs are affected by diseases such as diabetes and atherosclerosis. These microvessels may also be abnormal in people who have sickle cell disease. Stiffness of the red blood cells leads to problems in the microvessels of the heart and kidneys. However, these problems may not be detected until these organs are severely affected. Researchers want to study problems with microvessels in people with and without sickle cell disease. Objectives: \- To study how microvessels in the heart and other organs are affected by sickle cell disease. Eligibility:

• Individuals at least 18 years of age who have sickle cell disease.
• Healthy volunteers at least 18 years of age. Design:
• Participants will be screened with a physical exam and medical history. Blood and urine samples will be collected.
• All participants will have about 3 to 4 hours of testing for the study. Participants with sickle cell disease who are having a pain crisis at the time they enter the study may be asked to have the testing again when the crisis is over. The repeat testing will occur at least 4 weeks after the pain crisis ends.
• All participants will have the following tests:
• Blood draws to check kidney and liver function, and other blood tests
• Measure of blood flow in the brachial (upper arm) artery
• Heart ultrasound
• Ultrasound scans of arm muscles to study blood flow
• Ultrasound scans after taking vasodilators to increase blood flow
• Healthy volunteers will also have a magnetic resonance imaging scan. It will show blood flow in the heart. This scan will involve another dose of a vasodilator.

Conditions

Sickle Cell Disease

Keywords

Perfusion; Microvessels; Ultrasound; Sickle Cell Disease

Outcome Measures

Primary Outcomes (1)

• To use contrast-enhanced ultrasound to evaluate microvascular blood flow (MBF) and capillary RBC velocity (CBV) in skeletal muscle and the heart (at rest and during vasodilator stress) in patients with SCD in comparison to normal control patient...

Secondary Outcomes (2)

• To determine whether MBF and CBV worsen during pain crisis in patients with SCD

• To determine the relation between MBF and CBV and brachial artery flow or LV dysfunction.

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Adult subject age greater than or equal to 18 years
• Able to give written informed consent
• For SCD groups, must have confirmed diagnosis of sickle cell disease

You may not qualify if:

• Atrial fibrillation or other irregular rhythm that would preclude adequate image acquisition
• Subjects with a contraindication for the ultrasound contrast agent.
• Pregnant or lactating women
• Known obstructive coronary or peripheral vascular disease
• SCD subjects at steady-state must not have acute pain crisis requiring intravenous analgesics within the prior 4 weeks
• SCD subjects in crisis must be within 72 hours of hospital admission
• Subjects with contraindications to MRI scanning will complete all other procedures but will not undergo the MRI scan. Subjects with an estimated glomerular filtration rate of \<30 ml/min/1.73 m(2) will not receive gadolinium as per 2011 NHLBI gadolinium administration policy.
• Subjects with a contraindication to regadenson
• Any condition that in the clinical opinion of the investigators renders study procedures inadvisable.

Sponsors & Collaborators

• National Heart, Lung, and Blood Institute (NHLBI): lead

Study Sites (1)

National Institutes of Health Clinical Center, 9000 Rockville Pike

Bethesda, Maryland, 20892, United States

Location

Related Publications (4)

• Kato GJ, Hebbel RP, Steinberg MH, Gladwin MT. Vasculopathy in sickle cell disease: Biology, pathophysiology, genetics, translational medicine, and new research directions. Am J Hematol. 2009 Sep;84(9):618-25. doi: 10.1002/ajh.21475.

  PMID: 19610078 BACKGROUND

• Hebbel RP, Osarogiagbon R, Kaul D. The endothelial biology of sickle cell disease: inflammation and a chronic vasculopathy. Microcirculation. 2004 Mar;11(2):129-51.

  PMID: 15280088 BACKGROUND

• Morris CR, Kuypers FA, Larkin S, Vichinsky EP, Styles LA. Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome. J Pediatr Hematol Oncol. 2000 Nov-Dec;22(6):515-20. doi: 10.1097/00043426-200011000-00009.

  PMID: 11132219 BACKGROUND

• Sachdev V, Sidenko S, Wu MD, Minniti CP, Hannoush H, Brenneman CL, Waclawiw MA, Arai AE, Schechter AN, Kato GJ, Lindner JR. Skeletal and myocardial microvascular blood flow in hydroxycarbamide-treated patients with sickle cell disease. Br J Haematol. 2017 Nov;179(4):648-656. doi: 10.1111/bjh.14918. Epub 2017 Sep 7.

  PMID: 28880374 DERIVED

MeSH Terms

Conditions

Anemia, Sickle Cell

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

Study Officials

• Vandana Sachdev, M.D.

  National Heart, Lung, and Blood Institute (NHLBI)

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Time Perspective: PROSPECTIVE
• Sponsor Type: NIH
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: May 18, 2012
• First Posted: May 21, 2012
• Study Start: April 30, 2012
• Study Completion: October 25, 2017
• Last Updated: October 27, 2017

Record last verified: 2017-10-25

Locations

US (1)