Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia

Sickle Cell Disease Clinical Trial

Official title:

Determining the Prevalence and Prognosis of Secondary Pulmonary Hypertension in Adult Patients With Sickle Cell Anemia

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT00011648
• Other study ID #: 010088
• Secondary ID: 01-H-0088
• Status: Completed
• Start date: February 19, 2008

Study information

• Verified date: May 9, 2024
• Source: National Institutes of Health Clinical Center (CC)
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 986
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

• 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

Related Conditions & MeSH terms

• Anemia
• Anemia, Sickle Cell
• Hypertension
• Hypertension, Pulmonary
• Pulmonary Hypertension
• Sickle Cell Anemia
• Sickle Cell Disease

Locations

Country	Name	City	State
United States	National Institutes of Health Clinical Center	Bethesda	Maryland
United States	Howard University Hospital	Washington	District of Columbia

Sponsors (2)

Lead Sponsor	Collaborator
National Heart, Lung, and Blood Institute (NHLBI)	National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

Country where clinical trial is conducted

United States, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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	To determine whether genetic polymorphisms in candidate genes contribute to its development or response to treatment.		1 year

External Links

•   NIH Clinical Center Detailed Web Page