Hemodynamic predictors of mortality in adults with sickle cell disease

Alem Mehari, Shoaib Alam, Xin Tian, Michael J. Cuttica, Christopher F. Barnett, George Miles, Dihua Xu, Catherine Seamon, Patricia Adams-Graves, Oswaldo L. Castro, Caterina P. Minniti, Vandana Sachdev, James G. Taylor VI, Gregory J. Kato, Roberto F. Machado

Research output: Contribution to journalArticle

60 Citations (Scopus)

Abstract

Background: Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality, but no prior studies have evaluated quantitative relationships of mortality to physiological measures of pre- and postcapillary PH. Objectives: To identify risk factors associated with mortality and to estimate the expected survival in a cohort of patients with SCD with PH documented by right heart catheterization. Methods: Nine-year follow-up data (median, 4.7 yr) from the National Institutes of Health SCD PH screening study are reported. A total of 529 adults with SCD were screened by echocardiography between 2001 and 2010 with no exclusion criteria. Hemodynamic data were collected from 84 patients. PH was defined as mean pulmonary artery pressure (PAP) ≥ 25 mm Hg. Survival rates were estimated by the Kaplan-Meier method, and mortality risk factors were analyzed by the Cox proportional hazards regression. Measurements and Main Results: Specific hemodynamic variables were independently related to mortality: mean PAP (hazard ratio [HR], 1.61; 95% confidence interval [CI], 1.05-2.45 per 10 mm Hg increase; P = 0.027), diastolic PAP (HR, 1.83; 95% CI, 1.09-3.08 per 10mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19;95%CI, 1.23-3.89 per 10mm Hg increase; P = 0.008), transpulmonary gradient (HR, 1.78; 95% CI, 1.14- 2.79 per 10 mm Hg increase; P = 0.011), and pulmonary vascular resistance (HR, 1.44; 95% CI, 1.09-1.89 per Wood unit increase; P = 0.009) as risk factors for mortality. Conclusions: Mortality in adults with SCD and PH is proportional to the physiological severity of precapillary PH, demonstrating its prognostic and clinical relevance despite anemia-induced high cardiac output and less severely elevated pulmonary vascular resistance.

Original languageEnglish (US)
Pages (from-to)840-847
Number of pages8
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume187
Issue number8
DOIs
StatePublished - Apr 15 2013
Externally publishedYes

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Sickle Cell Anemia
Pulmonary Hypertension
Hemodynamics
Mortality
Pulmonary Artery
Confidence Intervals
Pressure
Vascular Resistance
High Cardiac Output
Pulmonary Wedge Pressure
National Institutes of Health (U.S.)
Cardiac Catheterization
Echocardiography
Anemia
Survival Rate
Survival

Keywords

  • Autopsy
  • Mortality
  • Pulmonary hypertension
  • Sickle cell

ASJC Scopus subject areas

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

Cite this

Mehari, A., Alam, S., Tian, X., Cuttica, M. J., Barnett, C. F., Miles, G., ... Machado, R. F. (2013). Hemodynamic predictors of mortality in adults with sickle cell disease. American Journal of Respiratory and Critical Care Medicine, 187(8), 840-847. https://doi.org/10.1164/rccm.201207-1222OC

Hemodynamic predictors of mortality in adults with sickle cell disease. / Mehari, Alem; Alam, Shoaib; Tian, Xin; Cuttica, Michael J.; Barnett, Christopher F.; Miles, George; Xu, Dihua; Seamon, Catherine; Adams-Graves, Patricia; Castro, Oswaldo L.; Minniti, Caterina P.; Sachdev, Vandana; Taylor VI, James G.; Kato, Gregory J.; Machado, Roberto F.

In: American Journal of Respiratory and Critical Care Medicine, Vol. 187, No. 8, 15.04.2013, p. 840-847.

Research output: Contribution to journalArticle

Mehari, A, Alam, S, Tian, X, Cuttica, MJ, Barnett, CF, Miles, G, Xu, D, Seamon, C, Adams-Graves, P, Castro, OL, Minniti, CP, Sachdev, V, Taylor VI, JG, Kato, GJ & Machado, RF 2013, 'Hemodynamic predictors of mortality in adults with sickle cell disease', American Journal of Respiratory and Critical Care Medicine, vol. 187, no. 8, pp. 840-847. https://doi.org/10.1164/rccm.201207-1222OC
Mehari, Alem ; Alam, Shoaib ; Tian, Xin ; Cuttica, Michael J. ; Barnett, Christopher F. ; Miles, George ; Xu, Dihua ; Seamon, Catherine ; Adams-Graves, Patricia ; Castro, Oswaldo L. ; Minniti, Caterina P. ; Sachdev, Vandana ; Taylor VI, James G. ; Kato, Gregory J. ; Machado, Roberto F. / Hemodynamic predictors of mortality in adults with sickle cell disease. In: American Journal of Respiratory and Critical Care Medicine. 2013 ; Vol. 187, No. 8. pp. 840-847.
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abstract = "Background: Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality, but no prior studies have evaluated quantitative relationships of mortality to physiological measures of pre- and postcapillary PH. Objectives: To identify risk factors associated with mortality and to estimate the expected survival in a cohort of patients with SCD with PH documented by right heart catheterization. Methods: Nine-year follow-up data (median, 4.7 yr) from the National Institutes of Health SCD PH screening study are reported. A total of 529 adults with SCD were screened by echocardiography between 2001 and 2010 with no exclusion criteria. Hemodynamic data were collected from 84 patients. PH was defined as mean pulmonary artery pressure (PAP) ≥ 25 mm Hg. Survival rates were estimated by the Kaplan-Meier method, and mortality risk factors were analyzed by the Cox proportional hazards regression. Measurements and Main Results: Specific hemodynamic variables were independently related to mortality: mean PAP (hazard ratio [HR], 1.61; 95{\%} confidence interval [CI], 1.05-2.45 per 10 mm Hg increase; P = 0.027), diastolic PAP (HR, 1.83; 95{\%} CI, 1.09-3.08 per 10mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19;95{\%}CI, 1.23-3.89 per 10mm Hg increase; P = 0.008), transpulmonary gradient (HR, 1.78; 95{\%} CI, 1.14- 2.79 per 10 mm Hg increase; P = 0.011), and pulmonary vascular resistance (HR, 1.44; 95{\%} CI, 1.09-1.89 per Wood unit increase; P = 0.009) as risk factors for mortality. Conclusions: Mortality in adults with SCD and PH is proportional to the physiological severity of precapillary PH, demonstrating its prognostic and clinical relevance despite anemia-induced high cardiac output and less severely elevated pulmonary vascular resistance.",
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AU - Mehari, Alem

AU - Alam, Shoaib

AU - Tian, Xin

AU - Cuttica, Michael J.

AU - Barnett, Christopher F.

AU - Miles, George

AU - Xu, Dihua

AU - Seamon, Catherine

AU - Adams-Graves, Patricia

AU - Castro, Oswaldo L.

AU - Minniti, Caterina P.

AU - Sachdev, Vandana

AU - Taylor VI, James G.

AU - Kato, Gregory J.

AU - Machado, Roberto F.

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N2 - Background: Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality, but no prior studies have evaluated quantitative relationships of mortality to physiological measures of pre- and postcapillary PH. Objectives: To identify risk factors associated with mortality and to estimate the expected survival in a cohort of patients with SCD with PH documented by right heart catheterization. Methods: Nine-year follow-up data (median, 4.7 yr) from the National Institutes of Health SCD PH screening study are reported. A total of 529 adults with SCD were screened by echocardiography between 2001 and 2010 with no exclusion criteria. Hemodynamic data were collected from 84 patients. PH was defined as mean pulmonary artery pressure (PAP) ≥ 25 mm Hg. Survival rates were estimated by the Kaplan-Meier method, and mortality risk factors were analyzed by the Cox proportional hazards regression. Measurements and Main Results: Specific hemodynamic variables were independently related to mortality: mean PAP (hazard ratio [HR], 1.61; 95% confidence interval [CI], 1.05-2.45 per 10 mm Hg increase; P = 0.027), diastolic PAP (HR, 1.83; 95% CI, 1.09-3.08 per 10mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19;95%CI, 1.23-3.89 per 10mm Hg increase; P = 0.008), transpulmonary gradient (HR, 1.78; 95% CI, 1.14- 2.79 per 10 mm Hg increase; P = 0.011), and pulmonary vascular resistance (HR, 1.44; 95% CI, 1.09-1.89 per Wood unit increase; P = 0.009) as risk factors for mortality. Conclusions: Mortality in adults with SCD and PH is proportional to the physiological severity of precapillary PH, demonstrating its prognostic and clinical relevance despite anemia-induced high cardiac output and less severely elevated pulmonary vascular resistance.

AB - Background: Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality, but no prior studies have evaluated quantitative relationships of mortality to physiological measures of pre- and postcapillary PH. Objectives: To identify risk factors associated with mortality and to estimate the expected survival in a cohort of patients with SCD with PH documented by right heart catheterization. Methods: Nine-year follow-up data (median, 4.7 yr) from the National Institutes of Health SCD PH screening study are reported. A total of 529 adults with SCD were screened by echocardiography between 2001 and 2010 with no exclusion criteria. Hemodynamic data were collected from 84 patients. PH was defined as mean pulmonary artery pressure (PAP) ≥ 25 mm Hg. Survival rates were estimated by the Kaplan-Meier method, and mortality risk factors were analyzed by the Cox proportional hazards regression. Measurements and Main Results: Specific hemodynamic variables were independently related to mortality: mean PAP (hazard ratio [HR], 1.61; 95% confidence interval [CI], 1.05-2.45 per 10 mm Hg increase; P = 0.027), diastolic PAP (HR, 1.83; 95% CI, 1.09-3.08 per 10mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19;95%CI, 1.23-3.89 per 10mm Hg increase; P = 0.008), transpulmonary gradient (HR, 1.78; 95% CI, 1.14- 2.79 per 10 mm Hg increase; P = 0.011), and pulmonary vascular resistance (HR, 1.44; 95% CI, 1.09-1.89 per Wood unit increase; P = 0.009) as risk factors for mortality. Conclusions: Mortality in adults with SCD and PH is proportional to the physiological severity of precapillary PH, demonstrating its prognostic and clinical relevance despite anemia-induced high cardiac output and less severely elevated pulmonary vascular resistance.

KW - Autopsy

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KW - Pulmonary hypertension

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