Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity

Roberto F. Machado, Robyn J. Barst, Nancy A. Yovetich, Kathryn L. Hassell, Gregory Kato, Victor R. Gordeuk, J. Simon R. Gibbs, Jane A. Little, Dean E. Schraufnagel, Lakshmanan Krishnamurti, Reda Girgis, Claudia R. Morris, Erika B. Rosenzweig, David B. Badesch, Sophie Lanzkron, Onyinye Onyekwere, Oswaldo L. Castro, Vandana Sachdev, Myron A. Waclawiw, Rob WoolsonJonathan C. Goldsmith, Mark T. Gladwin

Research output: Contribution to journalArticlepeer-review

165 Scopus citations


In adults with sickle cell disease (SCD), an increased tricuspid regurgitation velocity (TRV) by Doppler echocardiography is associated with increased morbidity and mortality. Although sildenafil has been shown to improve exercise capacity in patients with pulmonary arterial hypertension, it has not been evaluated in SCD. We therefore sought to determine whether sildenafil could improve exercise capacity in SCD patients with increased TRV and a low exercise capacity. A TRV ≥ 2.7 m/s and a 6-minute walk distance (6MWD) between 150 and 500 m were required for enrollment in this 16-week, double-blind, placebo-controlled sildenafil trial. After 74 of the screened subjects were randomized, the study was stopped early due to a higher percentage of subjects experiencing serious adverse events in the sildenafil arm (45% of sildenafil, 22% of placebo, P = .022). Subject hospitalization for pain was the predominant cause for this difference: 35% with sildenafil compared with 14% with placebo (P = .029). There was no evidence of a treatment effect on 6MWD (placebo-corrected effect -9 m; 95% confidence interval [95% CI] -56-38; P = .703), TRV (P = .503), or N-terminal pro-brain natriuretic peptide (P = .410). Sildenafil appeared to increase hospitalization rates for pain in patients with SCD. This study is registered at as NCT00492531.

Original languageEnglish (US)
Pages (from-to)855-864
Number of pages10
Issue number4
StatePublished - Jul 28 2011

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology


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