BACKGROUND: Recurrent vaso-occlusive episodes lead to progressive end-organ damage in patients with sickle cell disease. We sought to determine the prevalence of pulmonary hypertension in adult patients with sickle cell disease and to identify factors associated with this life-threatening complication. METHODS: Sixty patients (> or =18 years of age; mean [+/- SD] age, 37 +/- 13 years) followed at a University Medical Center were evaluated. They were selected by a systematic sampling of patients presenting to the clinic for routine follow-up visits. All enrolled subjects underwent a clinical examination, Doppler echocardiography, pulmonary function tests, and hematologic tests during a single visit. Pulmonary hypertension was defined using an age- and body mass index-adjusted nomogram. RESULTS: The prevalence of pulmonary hypertension was 30% (18/60). Ten patients had mild pulmonary hypertension (up to 44 mm Hg), 5 had moderate pulmonary hypertension (45 to 74 mm Hg), and 2 had severe pulmonary hypertension (> or =75 mm Hg). In a logistic regression model, both lower fetal hemoglobin level and lower systolic blood pressure were associated with the presence of pulmonary hypertension. CONCLUSION: We found that the prevalence of pulmonary hypertension in adult patients with sickle cell disease was substantial, particularly in those with lower levels of fetal hemoglobin and lower systolic blood pressure.
BACKGROUND: Recurrent vaso-occlusive episodes lead to progressive end-organ damage in patients with sickle cell disease. We sought to determine the prevalence of pulmonary hypertension in adult patients with sickle cell disease and to identify factors associated with this life-threatening complication. METHODS: Sixty patients (> or =18 years of age; mean [+/- SD] age, 37 +/- 13 years) followed at a University Medical Center were evaluated. They were selected by a systematic sampling of patients presenting to the clinic for routine follow-up visits. All enrolled subjects underwent a clinical examination, Doppler echocardiography, pulmonary function tests, and hematologic tests during a single visit. Pulmonary hypertension was defined using an age- and body mass index-adjusted nomogram. RESULTS: The prevalence of pulmonary hypertension was 30% (18/60). Ten patients had mild pulmonary hypertension (up to 44 mm Hg), 5 had moderate pulmonary hypertension (45 to 74 mm Hg), and 2 had severe pulmonary hypertension (> or =75 mm Hg). In a logistic regression model, both lower fetal hemoglobin level and lower systolic blood pressure were associated with the presence of pulmonary hypertension. CONCLUSION: We found that the prevalence of pulmonary hypertension in adult patients with sickle cell disease was substantial, particularly in those with lower levels of fetal hemoglobin and lower systolic blood pressure.
Authors: Robyn J Barst; Kamal K Mubarak; Roberto F Machado; Kenneth I Ataga; Raymond L Benza; Oswaldo Castro; Robert Naeije; Namita Sood; Paul S Swerdlow; Mariana Hildesheim; Mark T Gladwin Journal: Br J Haematol Date: 2010-02-17 Impact factor: 6.998
Authors: Claudia R Morris; Frans A Kuypers; Gregory J Kato; Lisa Lavrisha; Sandra Larkin; Titi Singer; Elliott P Vichinsky Journal: Ann N Y Acad Sci Date: 2005 Impact factor: 5.691
Authors: Nalini Raghavachari; Xiuli Xu; Amy Harris; Jose Villagra; Carolea Logun; Jennifer Barb; Michael A Solomon; Anthony F Suffredini; Robert L Danner; Gregory Kato; Peter J Munson; Sidney M Morris; Mark T Gladwin Journal: Circulation Date: 2007-03-12 Impact factor: 29.690
Authors: Vandana Sachdev; Roberto F Machado; Yukitaka Shizukuda; Yesoda N Rao; Stanislav Sidenko; Inez Ernst; Marilyn St Peter; Wynona A Coles; Douglas R Rosing; William C Blackwelder; Oswaldo Castro; Gregory J Kato; Mark T Gladwin Journal: J Am Coll Cardiol Date: 2007-01-16 Impact factor: 24.094
Authors: Mei-Sing Ong; Steve Abman; Eric D Austin; Jeffrey A Feinstein; Rachel K Hopper; Usha S Krishnan; Mary P Mullen; Marc D Natter; J Usha Raj; Erika B Rosenzweig; Kenneth D Mandl Journal: J Pediatr Date: 2019-06-05 Impact factor: 4.406