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Severity of pulmonary hypertension during vaso‐occlusive pain crisis and exercise in patients with sickle cell disease
Author(s) -
Machado Roberto F.,
Kyle Mack A.,
Martyr Sabrina,
Barnett Christopher,
MacArthur Peter,
Sachdev Vandana,
Ernst Inez,
Hunter Lori A.,
Coles Wya A.,
Nichols James P.,
Kato Gregory J.,
Gladwin Mark T.
Publication year - 2007
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2006.06417.x
Subject(s) - medicine , pulmonary hypertension , cardiology , vascular resistance , pulmonary artery , cohort , chest pain , hemodynamics
Summary Pulmonary hypertension is associated with sudden death and is a risk factor for mortality in adult patients with sickle cell disease. The high mortality despite only mild‐to‐moderate increases in pulmonary vascular resistance remains an unresolved paradox. Accordingly, little is known about the cardiovascular effects of stressors, such as vaso‐occlusive pain crisis (VOC) and exercise, which may acutely increase pulmonary pressures and impair right heart function. We therefore evaluated pulmonary artery pressures by echocardiogram in 25 patients with sickle cell disease in steady‐state and during VOC, and by right heart catheterisation with exercise in a second cohort of 21 patients to determine whether pulmonary hypertension worsens during acute cardiopulmonary stress. TRV increased during VOC ( P < 0·001), and the increased pulmonary pressures during VOC were associated with decreases in haemoglobin levels ( P < 0·001), and increases in lactate dehydrogenase ( P < 0·001) and plasma haemoglobin levels ( P = 0·03). During exercise stress performed during cardiac catheterisation, mean pulmonary artery pressures ( P < 0·001) and pulmonary vascular resistance increased ( P < 0·001) in all subjects. These data suggest that acute elevations in pulmonary pressures during VOC or exercise may contribute to morbidity and mortality in patients with sickle cell disease.