Cardiovascular complications and risk of death in sickle-cell disease

The Lancet

Volumn 387, Issue 10037, 2016, Pages 2565-2574

  Gladwin, Mark T ^a  

^a UNIVERSITY OF PITTSBURGH SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ANEMIA; CARDIOVASCULAR DISEASE; CARDIOVASCULAR MORTALITY; CARDIOVASCULAR RISK; CLINICAL FEATURE; DIASTOLIC DYSFUNCTION; HEMOLYSIS; HUMAN; ISCHEMIC HEART DISEASE; LUNG ARTERY PRESSURE; MORTALITY RISK; PATHOPHYSIOLOGY; PREMATURE MORTALITY; PRIORITY JOURNAL; PULMONARY HYPERTENSION; REVIEW; SICKLE CELL ANEMIA; SUDDEN DEATH; SYSTOLIC DYSFUNCTION; THROMBOEMBOLISM; COMPLICATION; DEATH, SUDDEN, CARDIAC; EARLY DIAGNOSIS; HEART DISEASES; HYPERTENSION, PULMONARY; MORTALITY; PHYSIOLOGY; RISK FACTOR;

ANEMIA, SICKLE CELL; DEATH, SUDDEN, CARDIAC; EARLY DIAGNOSIS; HEART DISEASES; HEMOLYSIS; HUMANS; HYPERTENSION, PULMONARY; RISK FACTORS;

EID: 84975044348     PISSN: 01406736     EISSN: 1474547X     Source Type: Journal    
DOI: 10.1016/S0140-6736(16)00647-4     Document Type: Review

References (97)

1. DC Rees, TN Williams, MT Gladwin Sickle-cell disease Lancet 376 2010 2018 2031
2. HF Bunn Pathogenesis and treatment of sickle cell disease N Engl J Med 337 1997 762 769
3. MT Gladwin, E Vichinsky Pulmonary complications of sickle cell disease N Engl J Med 359 2008 2254 2265
4. JG Taylor 6th, VG Nolan, L Mendelsohn, GJ Kato, MT Gladwin, MH Steinberg Chronic hyper-hemolysis in sickle cell anemia: association of vascular complications and mortality with less frequent vasoocclusive pain PLoS One 3 2008 e2095
5. GJ Kato, MT Gladwin, MH Steinberg Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes Blood Rev 21 2007 37 47
6. MT Gladwin, V Sachdev, ML Jison et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease N Engl J Med 350 2004 886 895
7. HF Bunn, DG Nathan, GJ Dover et al. Pulmonary hypertension and nitric oxide depletion in sickle cell disease Blood 116 2010 687 692
8. JD Belcher, CJ Bryant, J Nguyen et al. Transgenic sickle mice have vascular inflammation Blood 101 2003 3953 3959
9. UR Osarogiagbon, S Choong, JD Belcher, GM Vercellotti, MS Paller, RP Hebbel Reperfusion injury pathophysiology in sickle transgenic mice Blood 96 2000 314 320
10. P Connes, Y Lamarre, X Waltz et al. Haemolysis and abnormal haemorheology in sickle cell anaemia Br J Haematol 165 2014 564 572
11. GJ Kato, V McGowan, RF Machado et al. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease Blood 107 2006 2279 2285
12. M Nouraie, JS Lee, Y Zhang the Walk-PHASST Investigators and Patients The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe Haematologica 98 2013 464 472
13. FB Piel, SI Hay, S Gupta, DJ Weatherall, TN Williams Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mortality, and interventions PLoS Med 10 2013 e1001484
14. SD Grosse, I Odame, HK Atrash, DD Amendah, FB Piel, TN Williams Sickle cell disease in Africa: a neglected cause of early childhood mortality Am J Prev Med 41 2011 S398 S405
15. KI Ataga, CG Moore, S Jones et al. Pulmonary hypertension in patients with sickle cell disease: a longitudinal study Br J Haematol 134 2006 109 115
16. P Bartolucci, C Brugnara, A Teixeira-Pinto et al. Erythrocyte density in sickle cell syndromes is associated with specific clinical manifestations and hemolysis Blood 120 2012 3136 3141
17. TG Day, ER Drasar, T Fulford, CC Sharpe, SL Thein Association between hemolysis and albuminuria in adults with sickle cell anemia Haematologica 97 2012 201 205
18. CD Fitzhugh, N Lauder, JC Jonassaint et al. Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease Am J Hematol 85 2010 36 40
19. MT Gladwin, RJ Barst, JS Gibbs for the walk-PHaSST Investigators and Patients Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom PLoS One 9 2014 e99489
20. MT Gladwin, V Sachdev Cardiovascular abnormalities in sickle cell disease J Am Coll Cardiol 59 2012 1123 1133
21. A Mehari, S Alam, X Tian et al. Hemodynamic predictors of mortality in adults with sickle cell disease Am J Respir Crit Care Med 187 2013 840 847
22. EM Novelli, M Hildesheim, C Rosano et al. Elevated pulse pressure is associated with hemolysis, proteinuria and chronic kidney disease in sickle cell disease PLoS One 9 2014 e114309
23. OS Platt, DJ Brambilla, WF Rosse et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death N Engl J Med 330 1994 1639 1644
24. DR Powars, DD Elliott-Mills, L Chan et al. Chronic renal failure in sickle cell disease: risk factors, clinical course, and mortality Ann Intern Med 115 1991 614 620
25. SL Saraf, X Zhang, T Kanias et al. Haemoglobinuria is associated with chronic kidney disease and its progression in patients with sickle cell anaemia Br J Haematol 164 2014 729 739
26. JA Detterich, RM Kato, M Rabai, HJ Meiselman, TD Coates, JC Wood Chronic transfusion therapy improves but does not normalize systemic and pulmonary vasculopathy in sickle cell disease Blood 126 2015 703 710
27. R Pannu, J Zhang, R Andraws, A Armani, P Patel, P Mancusi-Ungaro Acute myocardial infarction in sickle cell disease: a systematic review Crit Pathw Cardiol 7 2008 133 138
28. J Pavlů, RE Ahmed, DP O'Regan, J Partridge, DC Lefroy, DM Layton Myocardial infarction in sickle-cell disease Lancet 369 2007 246
29. I Robard, N Mansencal, G Soulat, J Deblaise, R El Mahmoud, O Dubourg Myocardial infarction with normal coronary arteries in double heterozygous sickle-cell disease Int J Cardiol 180 2015 120 121
30. ES Klings, RF Machado, RJ Barst the American Thoracic Society Ad Hoc Committee on Pulmonary Hypertension of Sickle Cell Disease An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease Am J Respir Crit Care Med 189 2014 727 740
31. RF Machado, A Anthi, MH Steinberg the MSH Investigators N-terminal pro-brain natriuretic peptide levels and risk of death in sickle cell disease JAMA 296 2006 310 318
32. RF Machado, M Hildesheim, L Mendelsohn, AT Remaley, GJ Kato, MT Gladwin NT-pro brain natriuretic peptide levels and the risk of death in the cooperative study of sickle cell disease Br J Haematol 154 2011 512 520
33. A Mehari, MT Gladwin, X Tian, RF Machado, GJ Kato Mortality in adults with sickle cell disease and pulmonary hypertension JAMA 307 2012 1254 1256
34. CR Morris, GJ Kato, M Poljakovic et al. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease JAMA 294 2005 81 90
35. S Poludasu, K Ramkissoon, L Salciccioli, H Kamran, JM Lazar Left ventricular systolic function in sickle cell anemia: a meta-analysis J Card Fail 19 2013 333 341
36. V Sachdev, GJ Kato, JS Gibbs the Walk-PHASST Investigators Echocardiographic markers of elevated pulmonary pressure and left ventricular diastolic dysfunction are associated with exercise intolerance in adults and adolescents with homozygous sickle cell anemia in the United States and United Kingdom Circulation 124 2011 1452 1460
37. A Anthi, RF Machado, ML Jison et al. Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension Am J Respir Crit Care Med 175 2007 1272 1279
38. SB Roy, ML Bhatia, VS Mathur, S Virmani Hemodynamic Effects of Chronic Severe Anemia Circulation 28 1963 346 356
39. V Sachdev, RF Machado, Y Shizukuda et al. Diastolic dysfunction is an independent risk factor for death in patients with sickle cell disease J Am Coll Cardiol 49 2007 472 479
40. JI Hoffman Pulmonary hypertension in sickle cell disease N Engl J Med 365 2011 1647 1648
41. MR DeBaun, FD Armstrong, RC McKinstry, RE Ware, E Vichinsky, FJ Kirkham Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia Blood 119 2012 4587 4596
42. K Ohene-Frempong, SJ Weiner, LA Sleeper et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors Blood 91 1998 288 294
43. CD Reiter, X Wang, JE Tanus-Santos et al. Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease Nat Med 8 2002 1383 1389
44. RP Rother, L Bell, P Hillmen, MT Gladwin The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease JAMA 293 2005 1653 1662
45. LL Hsu, HC Champion, SA Campbell-Lee et al. Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability Blood 109 2007 3088 3098
46. MT Gladwin Deconstructing endothelial dysfunction: soluble guanylyl cyclase oxidation and the NO resistance syndrome J Clin Invest 116 2006 2330 2332
47. DK Kaul, XD Liu, ME Fabry, RL Nagel Impaired nitric oxide-mediated vasodilation in transgenic sickle mouse Am J Physiol Heart Circ Physiol 278 2000 H1799 H1806
48. MT Gladwin Revisiting the hyperhemolysis paradigm Blood 126 2015 695 696
49. C Donadee, NJ Raat, T Kanias et al. Nitric oxide scavenging by red blood cell microparticles and cell-free hemoglobin as a mechanism for the red cell storage lesion Circulation 124 2011 465 476
50. L Berra, R Pinciroli, CP Stowell et al. Autologous transfusion of stored red blood cells increases pulmonary artery pressure Am J Respir Crit Care Med 190 2014 800 807
51. CB Almeida, LE Souza, FC Leonardo et al. Acute hemolytic vascular inflammatory processes are prevented by nitric oxide replacement or a single dose of hydroxyurea Blood 126 2015 711 720
52. JN Milton, H Rooks, E Drasar the Walk-PHAAST Investigators Genetic determinants of haemolysis in sickle cell anaemia Br J Haematol 161 2013 270 278
53. S Ghosh, OA Adisa, P Chappa et al. Extracellular hemin crisis triggers acute chest syndrome in sickle mice J Clin Invest 123 2013 4809 4820
54. JD Belcher, C Chen, J Nguyen et al. Heme triggers TLR4 signaling leading to endothelial cell activation and vaso-occlusion in murine sickle cell disease Blood 123 2014 377 390
55. MT Gladwin, SF Ofori-Acquah Erythroid DAMPs drive inflammation in SCD Blood 123 2014 3689 3690
56. J Villagra, S Shiva, LA Hunter, RF Machado, MT Gladwin, GJ Kato Platelet activation in patients with sickle disease, hemolysis-associated pulmonary hypertension, and nitric oxide scavenging by cell-free hemoglobin Blood 110 2007 2166 2172
57. N Cardenes, C Corey, L Geary et al. Platelet bioenergetic screen in sickle cell patients reveals mitochondrial complex V inhibition, which contributes to platelet activation Blood 123 2014 2864 2872
58. CC Helms, M Marvel, W Zhao et al. Mechanisms of hemolysis-associated platelet activation J Thromb Haemost 11 2013 2148 2154
59. KI Ataga Hypercoagulability and thrombotic complications in hemolytic anemias Haematologica 94 2009 1481 1484
60. YC Lai, KC Potoka, HC Champion, AL Mora, MT Gladwin Pulmonary arterial hypertension: the clinical syndrome Circ Res 115 2014 115 130
61. MM Hoeper, HJ Bogaard, R Condliffe et al. Definitions and diagnosis of pulmonary hypertension J Am Coll Cardiol 62 suppl 2013 D42 D50
62. G Simonneau, MA Gatzoulis, I Adatia et al. Updated clinical classification of pulmonary hypertension J Am Coll Cardiol 62 suppl 2013 D34 D41
63. O Castro, M Hoque, BD Brown Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival Blood 101 2003 1257 1261
64. F Parent, D Bachir, J Inamo et al. A hemodynamic study of pulmonary hypertension in sickle cell disease N Engl J Med 365 2011 44 53
65. GH Fonseca, R Souza, VM Salemi, CV Jardim, SF Gualandro Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease Eur Respir J 39 2012 112 118
66. LM De Castro, JC Jonassaint, FL Graham, A Ashley-Koch, MJ Telen Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes Am J Hematol 83 2008 19 25
67. D Lorch, D Spevack, J Little An elevated estimated pulmonary arterial systolic pressure, whenever measured, is associated with excess mortality in adults with sickle cell disease Acta Haematol 125 2011 225 229
68. MC Caughey, C Poole, KI Ataga, AL Hinderliter Estimated pulmonary artery systolic pressure and sickle cell disease: a meta-analysis and systematic review Br J Haematol 170 2015 416 424
69. AO Dosunmu, TM Balogun, OO Adeyeye et al. Prevalence of pulmonary hypertension in sickle cell anaemia patients of a tertiary hospital in Nigeria Niger Med J 55 2014 161 165
70. ZY Aliyu, A Suleiman, E Attah et al. NT-proBNP as a marker of cardiopulmonary status in sickle cell anaemia in Africa Br J Haematol 150 2010 102 107
71. ZY Aliyu, V Gordeuk, V Sachdev et al. Prevalence and risk factors for pulmonary artery systolic hypertension among sickle cell disease patients in Nigeria Am J Hematol 83 2008 485 490
72. R Marouf, N Behbehani, M Zubaid et al. Transthoracic echocardiography and 6-minute walk test in Kuwaiti sickle cell disease patients Med Princ Pract 23 2014 212 217
73. RP Naik, MB Streiff, C Haywood Jr, JB Segal, S Lanzkron Venous thromboembolism incidence in the Cooperative Study of Sickle Cell Disease J Thromb Haemost 12 2014 2010 2016
74. GL Yung, RN Channick, PF Fedullo et al. Successful pulmonary thromboendarterectomy in two patients with sickle cell disease Am J Respir Crit Care Med 157 1998 1690 1693
75. HA Ghofrani, AM D'Armini, F Grimminger the CHEST-1 Study Group Riociguat for the treatment of chronic thromboembolic pulmonary hypertension N Engl J Med 369 2013 319 329
76. RF Machado, RJ Barst, NA Yovetich the walk-PHaSST Investigators and Patients Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity Blood 118 2011 855 864
77. MC Caldas, ZA Meira, MM Barbosa Evaluation of 107 patients with sickle cell anemia through tissue Doppler and myocardial performance index J Am Soc Echocardiogr 21 2008 1163 1167
78. JS Hankins, MB McCarville, CM Hillenbrand et al. Ventricular diastolic dysfunction in sickle cell anemia is common but not associated with myocardial iron deposition Pediatr Blood Cancer 55 2010 495 500
79. MC Johnson, FJ Kirkham, S Redline et al. Left ventricular hypertrophy and diastolic dysfunction in children with sickle cell disease are related to asleep and waking oxygen desaturation Blood 116 2010 16 21
80. F Bode-Thomas, HI Hyacinth, O Ogunkunle, A Omotoso Myocardial ischaemia in sickle cell anaemia: evaluation using a new scoring system Ann Trop Paediatr 31 2011 67 74
81. AJ Deymann, KK Goertz Myocardial infarction and transient ventricular dysfunction in an adolescent with sickle cell disease Pediatrics 111 2003 E183 E187
82. NC Dang, C Johnson, M Eslami-Farsani, LJ Haywood Myocardial injury or infarction associated with fat embolism in sickle cell disease: a report of three cases with survival Am J Hematol 80 2005 133 136
83. GJ Kato, MT Gladwin Evolution of novel small-molecule therapeutics targeting sickle cell vasculopathy JAMA 300 2008 2638 2646
84. S Yuditskaya, A Tumblin, GT Hoehn et al. Proteomic identification of altered apolipoprotein patterns in pulmonary hypertension and vasculopathy of sickle cell disease Blood 113 2009 1122 1128
85. S Norris, CS Johnson, LJ Haywood Sickle cell anemia: does myocardial ischemia occur during crisis? J Natl Med Assoc 83 1991 209 213
86. M de Montalembert, C Maunoury, P Acar, V Brousse, D Sidi, G Lenoir Myocardial ischaemia in children with sickle cell disease Arch Dis Child 89 2004 359 362
87. P Acar, C Maunoury, M de Montalembert, Y Dulac Abnormalities of myocardial perfusion in sickle cell disease in childhood: a study of myocardial scintigraphy (in French) Arch Mal Coeur Vaiss 96 2003 507 510
88. MA Tanner, MA Westwood, DJ Pennell Myocardial infarction following sickle cell chest syndrome Br J Haematol 134 2006 2
89. Z Khalique, J Pavlu, D Lefroy, M Layton Erythrocytapheresis in the prevention of recurrent myocardial infarction in sickle cell disease Am J Hematol 85 2010 91
90. WH Johnson Jr, RB McCrary, VN Mankad Transient left ventricular dysfunction in childhood sickle cell disease Pediatr Cardiol 20 1999 221 223
91. EA Manci, DE Culberson, YM Yang the Investigators of the Cooperative Study of Sickle Cell Disease Causes of death in sickle cell disease: an autopsy study Br J Haematol 123 2003 359 365
92. A Gray, EN Anionwu, SC Davies, M Brozovic Patterns of mortality in sickle cell disease in the United Kingdom J Clin Pathol 44 1991 459 463
93. AK Haque, S Gokhale, BA Rampy, P Adegboyega, A Duarte, MJ Saldana Pulmonary hypertension in sickle cell hemoglobinopathy: a clinicopathologic study of 20 cases Hum Pathol 33 2002 1037 1043
94. A Mekontso Dessap, R Leon, A Habibi et al. Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease Am J Respir Crit Care Med 177 2008 646 653
95. A Maisel, H Friedman, L Flint, M Koshy, R Prabhu Continuous electrocardiographic monitoring in patients with sickle-cell anemia during pain crisis Clin Cardiol 6 1983 339 344
96. F Akgül, E Seyfeli, I Melek et al. Increased QT dispersion in sickle cell disease: effect of pulmonary hypertension Acta Haematol 118 2007 1 6
97. B Upadhya, W Ntim, R Brandon Stacey et al. Prolongation of QTc intervals and risk of death among patients with sickle cell disease Eur J Haematol 91 2013 170 178