Secondary Pulmonary Hypertension in Adults With Sickle Cell Anemia

Completed

• Conditions: Pulmonary Hypertension; Sickle Cell Anemia; Sickle Cell Disease

• Registration Number: NCT00011648
• Lead Sponsor: National Heart, Lung, and Blood Institute (NHLBI)

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.

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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 Timeline

• Study First Submitted: 2001-02-24
• Study First Submitted QC: 2001-02-24
• Study First Posted: 2001-02-26
• Study Start: 2008-02-19
• Status Verified: 2025-02-18
• Last Update Submitted: 2025-06-04
• Last Update Posted: 2025-06-05

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 986

Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
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

Name	Time	Method
To determine whether genetic polymorphisms in candidate genes contribute to its development or response to treatment.	1 year

Trial Locations

Locations (2)

Howard University Hospital; 🇺🇸 Washington, District of Columbia, United States

National Institutes of Health Clinical Center; 🇺🇸 Bethesda, Maryland, United States