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Pulmonary hypertension in sickle cell disease
Kenneth I Ataga1, Namita Sood, Guy De Gent
1Department of Medicine, University of North Carolina, Chapel Hill, North Carolina, USA. kataga@med.unc.edu
The American Journal of Medicine
|October 27, 2004
Summary
Pulmonary hypertension affects 30% of adult sickle cell disease patients. Lower fetal hemoglobin and systolic blood pressure are linked to this serious complication.
Area of Science:
- Cardiology
- Hematology
- Pulmonology
Background:
- Sickle cell disease (SCD) causes progressive end-organ damage through recurrent vaso-occlusive episodes.
- Pulmonary hypertension (PH) is a life-threatening complication in SCD patients.
- Identifying PH prevalence and associated factors in adults with SCD is crucial.
Purpose of the Study:
- To determine the prevalence of pulmonary hypertension in adult patients with sickle cell disease.
- To identify factors associated with pulmonary hypertension in this population.
Main Methods:
- Sixty adult SCD patients (age >= 18) were systematically sampled.
- Evaluations included clinical examination, Doppler echocardiography, pulmonary function tests, and hematologic tests.
- Pulmonary hypertension was defined using an age- and body mass index-adjusted nomogram.
Main Results:
- The prevalence of pulmonary hypertension was 30% (18/60) among adult SCD patients.
- PH severity ranged from mild (10 patients) to moderate (5 patients) and severe (2 patients).
- Lower fetal hemoglobin levels and lower systolic blood pressure were significantly associated with PH.
Conclusions:
- Pulmonary hypertension is a substantial complication in adult sickle cell disease patients.
- Lower fetal hemoglobin and systolic blood pressure are key indicators for PH risk in SCD.
- These findings highlight the need for monitoring and early intervention for PH in SCD.