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 10 mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19; 95% CI, 1.23-3.89 per 10 mm 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.
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 10 mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19; 95% CI, 1.23-3.89 per 10 mm 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.
Authors: H Franklin Bunn; David G Nathan; George J Dover; Robert P Hebbel; Orah S Platt; Wendell F Rosse; Russell E Ware Journal: Blood Date: 2010-04-15 Impact factor: 22.113
Authors: Gérald Simonneau; Ivan M Robbins; Maurice Beghetti; Richard N Channick; Marion Delcroix; Christopher P Denton; C Gregory Elliott; Sean P Gaine; Mark T Gladwin; Zhi-Cheng Jing; Michael J Krowka; David Langleben; Norifumi Nakanishi; Rogério Souza Journal: J Am Coll Cardiol Date: 2009-06-30 Impact factor: 24.094
Authors: Christopher D Reiter; Xunde Wang; Jose E Tanus-Santos; Neil Hogg; Richard O Cannon; Alan N Schechter; Mark T Gladwin Journal: Nat Med Date: 2002-11-11 Impact factor: 53.440
Authors: Stephen C Mathai; Laura K Hummers; Hunter C Champion; Fredrick M Wigley; Ari Zaiman; Paul M Hassoun; Reda E Girgis Journal: Arthritis Rheum Date: 2009-02
Authors: D Mukerjee; D St George; B Coleiro; C Knight; C P Denton; J Davar; C M Black; J G Coghlan Journal: Ann Rheum Dis Date: 2003-11 Impact factor: 19.103
Authors: Emelia J Benjamin; Michael J Blaha; Stephanie E Chiuve; Mary Cushman; Sandeep R Das; Rajat Deo; Sarah D de Ferranti; James Floyd; Myriam Fornage; Cathleen Gillespie; Carmen R Isasi; Monik C Jiménez; Lori Chaffin Jordan; Suzanne E Judd; Daniel Lackland; Judith H Lichtman; Lynda Lisabeth; Simin Liu; Chris T Longenecker; Rachel H Mackey; Kunihiro Matsushita; Dariush Mozaffarian; Michael E Mussolino; Khurram Nasir; Robert W Neumar; Latha Palaniappan; Dilip K Pandey; Ravi R Thiagarajan; Mathew J Reeves; Matthew Ritchey; Carlos J Rodriguez; Gregory A Roth; Wayne D Rosamond; Comilla Sasson; Amytis Towfighi; Connie W Tsao; Melanie B Turner; Salim S Virani; Jenifer H Voeks; Joshua Z Willey; John T Wilkins; Jason Hy Wu; Heather M Alger; Sally S Wong; Paul Muntner Journal: Circulation Date: 2017-01-25 Impact factor: 29.690
Authors: Nargues A Weir; Rehan Saiyed; Shoaib Alam; Anna Conrey; Himanshu D Desai; M Patricia George; Jennifer H Keeley; Elizabeth S Klings; Alem Mehari; James G Taylor; Caterina P Minniti; Gregory J Kato Journal: Haematologica Date: 2017-01-25 Impact factor: 9.941
Authors: Enrico M Novelli; Lynda Little-Ihrig; Heather E Knupp; Natasha M Rogers; Mingyi Yao; Jeffrey J Baust; Daniel Meijles; Claudette M St Croix; Mark A Ross; Patrick J Pagano; Evan R DeVallance; George Miles; Karin P Potoka; Jeffrey S Isenberg; Mark T Gladwin Journal: Am J Physiol Lung Cell Mol Physiol Date: 2019-03-20 Impact factor: 5.464
Authors: Rosario Di Maggio; Anna Conrey; Tiffani N Taylor; Robert J Lederman; Toby Rogers; Jim Nichols; Shenikqua Bouges; Caterina P Minniti; Gregory J Kato; Arun S Shet; Matthew M Hsieh Journal: Br J Haematol Date: 2018-10-23 Impact factor: 6.998