Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension

Anastasia Anthi, Roberto F. Machado, Maria L. Jison, Angelo M. Taveira-DaSilva, Lewis J. Rubin, Lori Hunter, Christian J. Hunter, Wynona Coles, James Nichols, Nilo A. Avila, Vandana Sachdev, Clara C. Chen, Mark T. Gladwin

Research output: Contribution to journalArticle

182 Scopus citations

Abstract

Rationale: Although pulmonary hypertension (PH) is a common complication of sickle cell disease (SCD) associated with high mortality, there exist few data characterizing hemodynamics and cardiopulmonary function in this population. Objectives: To characterize hemodynamics and cardiopulmonary function in patients with SCD with and without PH. Methods: Patients with SCD with PH (n = 26) were compared with control subjects with SCD but without PH (n = 17), matched for age, hemoglobin levels, and fetal hemoglobin levels. Measurements and Main Results: Upon catheterization, 54% of the patients with PH had pulmonary arterial hypertension, and 46% had pulmonary venous hypertension. When compared with control subjects, patients with PH exhibited lower six-minute-walk distance (435 ± 31 vs. 320 ± 20 m, p = 0.002) and oxygen consumption (50 ± 3% vs. 41 ± 2% of predicted, p = 0.02), and also had mild restrictive lung disease and more perfusion abnormalities on radionuclide lung scans. The six-minute-walk distance in this population inversely correlated with tricuspid regurgitant jet velocity (r = -0.55, p < 0.001), and mean pulmonary artery pressure (r = -0.57, p < 0.001), and directly correlated with maximal oxygen consumption (r = 0.49, p = 0.004), even after adjustment for hemoglobin, supporting an independent contribution of increasing pulmonary artery pressures to loss of exercise capacity. Conclusions: Patients with SCD-associated PH have both pulmonary arterial and venous PH associated with severe limitations in exercise capacity, likely compounded by interstitial lung fibrosis and severe anemia. These data support the use of the six-minute-walk distance as an index of PH and cardiopulmonary function in patients with SCD.

Original languageEnglish (US)
Pages (from-to)1272-1279
Number of pages8
JournalAmerican journal of respiratory and critical care medicine
Volume175
Issue number12
DOIs
StatePublished - Jun 15 2007
Externally publishedYes

Keywords

  • Echocardiogram
  • Hemodynamics
  • Pulmonary hypertension
  • Sickle cell disease
  • Six-minute walk

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

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    Anthi, A., Machado, R. F., Jison, M. L., Taveira-DaSilva, A. M., Rubin, L. J., Hunter, L., Hunter, C. J., Coles, W., Nichols, J., Avila, N. A., Sachdev, V., Chen, C. C., & Gladwin, M. T. (2007). Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension. American journal of respiratory and critical care medicine, 175(12), 1272-1279. https://doi.org/10.1164/rccm.200610-1498OC