Cardiovascular Complications of Sickle Cell Disease

NCT01044901 | Completed Results

• 1 other identifier: 16653A
• Study Type: observational
• Target: 51
• Locations: 1 country
• Sites: 1

Brief Summary

In this research study, we are using heart imaging exams and blood testing, in order to gain an improved understanding of the pulmonary (lung) hypertension and cardiovascular (heart) complications that often occur in sickle cell patients. Information gathered from the healthy volunteers that participate in this study will be compared to information from the sickle cell patients in this study in order to help further our understanding.

Conditions

Sickle Cell Disease

Keywords

Sickle Cell Disease; Cardiac magnetic resonance imaging; Coronary Disease; Pulmonary Hypertension

Outcome Measures

Primary Outcomes (19)

• MRI Parameter - LVEDVi, mL/cm2 (Measured Using Method of Disks, Controls Serve as Normal Ranges)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - LVESVi, mL/cm2 - (Measured Using Method of Disks, Controls Serve as Normal Ranges)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - LV Mass Index, g/cm2, (Measured Using Method of Disks, Controls Serve as Normal Ranges)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - RVEDVi, mL/cm2, (Measured Using Method of Disks, Controls Serve as Normal Ranges)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - RVESVi, mL/cm2, (Measured Using Method of Disks, Controls Serve as Normal Ranges)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - LAi, mL/cm2, (Measured Using Method of Disks, Controls Serve as Normal Ranges)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - RAi, mL/cm2, (Measured Using Method of Disks, Controls Serve as Normal Ranges)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - LVEF, %, (Measured Using Method of Disks, Controls Serve as Normal Ranges)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - RVEF, %, (Measured Using Method of Disks, Controls Serve as Normal Ranges)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter at baseline.

• MRI Parameter - Late Gadolinium Enhancement, Performed Via Visual Inspection, Normally None Should be Present

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - Myocardial T2-star, ms, Performed Using Decay Curves (Normal >20ms)

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - Hepatic T2-star, ms, Performed Using Decay Curves, Normal >18ms

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter at baseline.

• MRI Parameter - Myocardial Perfusion Reserve Index, Measured Using Upslope Technique. Control Subjects Available for Normal Ranges

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - Diastolic Dysfunction, Determined According to American Society of Echocardiography Guidelines

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - Lateral E/e', Measured Using Doppler Echo. Controls Available as Normal Ranges

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - Augmentation Pressure, See Controls for Normal Ranges

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - Augmentation Index, See Control Subjects for Normal Ranges

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - Systemic Systolic Blood Pressure

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

• MRI Parameter - Systemic Diastolic Blood Pressure, mm Hg

  Comprehensively and quantitatively characterized the cardiopulmonary complications of SCD and gained an improved understanding of the pathophysiology of pulmonary hypertension and diastolic dysfunction in patients with Sickle Cell Disease.

  Parameter measured at baseline.

Secondary Outcomes (1)

• Genome-Wide Gene Expression and Targeted Genetic Polymorphisms in SCD Patients Linked to a Quantitative Noninvasive-based PH Phenotype.

  median follow up 3 years

Study Arms (2)

Subjects with Sickle Cell Disease (SCD)

38 clinically stable black patients with Sickle Cell Disease (SCD) (including individuals with hemoglobin SS, SC, and β-thalassemia demonstrated by high-performance liquid chromatographic separation or gel electrophoresis).

Procedure: MRI, Transthoracic Echocardiography, tonometry, EKG

Healthy Volunteers

13 healthy control subjects were frequency matched to patients with SCD on age, sex, and race.

Procedure: MRI, Transthoracic Echocardiography, tonometry, EKG

Interventions

MRI, Transthoracic Echocardiography, tonometry, EKG PROCEDURE

Unless contraindicated, subjects will receive Regadenoson and Gadolinium contrast agent during the Cardiac magnetic resonance. The tonometer, EKG, and echo are non-invasive procedures.

Also known as: electrocardiography, Cardiac magnetic resonance, Doppler trans-thoracic echocardiography

Healthy Volunteers; Subjects with Sickle Cell Disease (SCD)

Eligibility Criteria

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

Study Population

Subjects will be recruited from clinics at the University of Chicago as well as from the community via flyers posted at the University of Chicago and a web site posted on the University of Chicago Medical Center web site.

You may qualify if:

• Patients must be 18+
• Patients who were diagnosed with SCD confirmed by high-pressure liquid chromatography or hemoglobin electrophoresis will be eligible for the study
• Only patients in stable condition will be included
• Patients receiving transfusions will not be excluded

You may not qualify if:

• Patients with vaso-occlusive crises or an episode of acute chest syndrome within the previous four weeks (after 4 weeks have passed, the patients may be re-evaluated for eligibility)
• Patients with high degree heart block; active, hemodynamically significant, ventricular arrhythmias; unstable coronary syndromes; history of myocardial infarction within 1 month of the study.
• Contraindications to gadolinium-enhanced magnetic resonance examination such as severe claustrophobia, Pacemaker, defibrillators, cerebral aneurysm clips, or neurostimulator.
• Pregnancy
• Patients with sinus node dysfunction

Sponsors & Collaborators

• University of Chicago: lead

Study Sites (1)

University of Chicago Medical Center

Chicago, Illinois, 60430, United States

Location

Related Publications (1)

• Desai AA, Patel AR, Ahmad H, Groth JV, Thiruvoipati T, Turner K, Yodwut C, Czobor P, Artz N, Machado RF, Garcia JGN, Lang RM. Mechanistic insights and characterization of sickle cell disease-associated cardiomyopathy. Circ Cardiovasc Imaging. 2014 May;7(3):430-437. doi: 10.1161/CIRCIMAGING.113.001420. Epub 2014 Mar 27.

  PMID: 24676783 RESULT

Biospecimen

Retention: SAMPLES WITH DNA

We will study blood samples for chemicals related to sickle cell and heart and lung disease, and will be evaluating DNA for purposes of the study.

MeSH Terms

Conditions

Anemia, Sickle Cell; Coronary Disease; Hypertension, Pulmonary

Interventions

Magnetic Resonance Spectroscopy; Manometry

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; Myocardial Ischemia; Heart Diseases; Cardiovascular Diseases; Vascular Diseases; Lung Diseases; Respiratory Tract Diseases; Hypertension

Intervention Hierarchy (Ancestors)

Spectrum Analysis; Chemistry Techniques, Analytical; Investigative Techniques

Limitations and Caveats

Due to small sample size, caution should be considered in applying these results to all patients with SCD, in particular to those with genotypes outside of hemoglobin and those with a recent or ongoing crisis. Not all patients had evaluable data sets for all of the cardiovascular tests, due to inadequate venous access, frequently encountered in patients with SCD. Finally, our follow-up period is currently too short to make meaningful comments on outcomes such as mortality and functional status.

Results Point of Contact

• Title: Dr. Amit Patel, MD
• Organization: The University of Chicago

apatel2@medicine.bsd.uchicago.edu

773-702-9461

Study Officials

• Amit R Patel, M.D.

  University of Chicago

  PRINCIPAL INVESTIGATOR

Publication Agreements

• PI is Sponsor Employee: No
• Restrictive Agreement: No

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: January 6, 2010
• First Posted: January 8, 2010
• Study Start: March 1, 2009
• Primary Completion: February 1, 2021
• Study Completion: February 1, 2021
• Last Updated: February 23, 2022
• Results First Posted: September 24, 2021

Record last verified: 2022-02

Data Sharing

• IPD Sharing: Will not share

Locations

US (1)