Pulmonary hypertension associated with sickle cell disease: Pathophysiology and rationale for treatment

Lung

Volumn 186, Issue 4, 2008, Pages 247-254

  Benza, Raymond L ^a  

^a UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

Author keywords

Endothelin receptor antagonist; Pulmonary hypertension; Sickle cell disease

Indexed keywords

AMBRISENTAN; AMINO TERMINAL PRO BRAIN NATRIURETIC PEPTIDE; ARGININE; BOSENTAN; BRAIN NATRIURETIC PEPTIDE; ENDOTHELIN 1; ENDOTHELIN 2; ENDOTHELIN 3; ENDOTHELIN RECEPTOR ANTAGONIST; ILOPROST; NITRIC OXIDE; PROSTACYCLIN; SILDENAFIL; UNIPROST;

ABDOMINAL DISCOMFORT; ARTICLE; BLOOD VESSEL INJURY; CHRONIC LIVER DISEASE; CONTINUOUS INFUSION; COUGHING; DIARRHEA; DRUG MEGADOSE; FLUSHING; HEADACHE; HEMOGLOBIN BLOOD LEVEL; HEMOLYSIS; HUMAN; INJECTION SITE PAIN; IRON OVERLOAD; LIVER TOXICITY; NOSE CONGESTION; OXIDATIVE STRESS; PAIN; PATHOPHYSIOLOGY; PATIENT COMPLIANCE; PRIAPISM; PRIORITY JOURNAL; PULMONARY HYPERTENSION; RASH; SEPSIS; SICKLE CELL ANEMIA; SIDE EFFECT; THROMBOEMBOLISM; THROMBOSIS; VISUAL DISORDER;

ANEMIA, SICKLE CELL; ANTIHYPERTENSIVE AGENTS; HUMANS; HYPERTENSION, PULMONARY; TREATMENT OUTCOME;

EID: 47549104449     PISSN: 03412040     EISSN: None     Source Type: Journal    
DOI: 10.1007/s00408-008-9092-8     Document Type: Article

References (56)

1. G Simonneau N Galie LJ Rubin D Langleben W Seeger G Domenighetti S Gibbs D Lebrec R Speich M Beghetti S Rich A Fishman 2004 Clinical classification of pulmonary hypertension J Am Coll Cardiol 43 5S 12S
2. OS Platt DJ Brambilla WF Rosse PF Milner O Castro MH Steinberg PP Klug 1994 Mortality in sickle cell disease. Life expectancy and risk factors for early death N Engl J Med 330 1639 1644
3. LM Graham 2004 The effect of sickle cell disease on the lung Clin Pulm Med 11 369 378
4. E Bossone BD Bodini A Mazza L Allegra 2005 Pulmonary arterial hypertension: the key role of echocardiography Chest 127 1836 1843
5. O Castro M Hoque BD Brown 2003 Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival Blood 101 1257 1261
6. MT Gladwin V Sachdev ML Jison Y Shizukuda JF Plehn K Minter B Brown WA Coles JS Nichols I Ernst LA Hunter WC Blackwelder AN Schechter GP Rodgers O Castro FP Ognibene 2004 Pulmonary hypertension as a risk factor for death in patients with sickle cell disease N Engl J Med 350 886 895
7. KR Minter MT Gladwin 2001 Pulmonary complications of sickle cell anemia. A need for increased recognition, treatment, and research Am J Respir Crit Care Med 164 2016 2019
8. M McGoon D Gutterman V Steen R Barst DC McCrory TA Fortin JE Loyd American College of Chest Physicians 2004 Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines Chest 126 14S 34S
9. JK Graham M Mosunjac RL Hanzlick 2007 Sickle cell lung disease and sudden death: a retrospective/prospective study of 21 autopsy cases and literature review Am J Forensic Med Pathol 28 168 172
10. CA Hillery JA Panepinto 2004 Pathophysiology of stroke in sickle cell disease Microcirculation 11 195 208
11. KI Ataga N Sood G De Gent E Kelly AG Henderson S Jones D Strayhorn A Lail S Lieff EP Orringer 2004 Pulmonary hypertension in sickle cell disease Am J Med 117 665 669
12. RF Machado MT Gladwin 2005 Chronic sickle cell lung disease: new insights into the diagnosis, pathogenesis and treatment of pulmonary hypertension Br J Haematol 129 449 464
13. A Anthi RF Machado ML Jison AM Taveira-Dasilva LJ Rubin L Hunter CJ Hunter W Coles J Nichols NA Avila V Sachdev CC Chen MT Gladwin 2007 Hemodynamic and functional assessment of patients with sickle cell disease and pulmonary hypertension Am J Respir Crit Care Med 175 1272 1279
14. V Sachdev RF Machado Y Shizukuda YN Rao S Sidenko I Ernst M St Peter WA Coles DR Rosing WC Blackwelder O Castro GJ Kato MT Gladwin 2007 Diastolic dysfunction is an independent risk factor for death in patients with sickle cell disease J Am Coll Cardiol 49 472 479
15. KI Ataga CG Moore S Jones O Olajide D Strayhorn A Hinderliter EP Orringer 2006 Pulmonary hypertension in patients with sickle cell disease: a longitudinal study Br J Haematol 134 109 115
16. RI Liem LT Young AA Thompson 2007 Tricuspid regurgitant jet velocity is associated with hemolysis in children and young adults with sickle cell disease evaluated for pulmonary hypertension Haematologica 92 1549 1552
17. SC Nelson BB Adade EA McDonough KL Moquist JM Hennessy 2007 High prevalence of pulmonary hypertension in children with sickle cell disease J Pediatr Hematol Oncol 29 334 337
18. A El-Beshlawy E Abd El Raouf F Mostafa M Talaat H Isma'eel E Aoun AV Hoffbrand A Taher 2006 Diastolic dysfunction and pulmonary hypertension in sickle cell anemia: is there a role for L-carnitine treatment? Acta Haematol 115 91 96
19. MN Suell LI Bezold MF Okcu DH Mahoney Jr F Shardonofsky BU Mueller 2005 Increased pulmonary artery pressures among adolescents with sickle cell disease J Pediatr Hematol Oncol 27 654 658
20. RW Hagar JG Michlitsch J Gardner EP Vichinsky CR Morris 2008 Clinical differences between children and adults with pulmonary hypertension and sickle cell disease Br J Haematol 140 104 112
21. CR Morris ST Singer MC Walters 2006 Clinical hemoglobinopathies: iron, lungs and new blood Curr Opin Hematol 13 407 418
22. A Aessopos M Kati D Farmakis 2007 Heart disease in thalassemia intermedia: a review of the underlying pathophysiology Haematologica 92 658 665
23. E Voskaridou G Tsetsos A Tsoutsias E Spyropoulou D Christoulas E Terpos 2007 Pulmonary hypertension in patients with sickle cell/beta thalassemia: incidence and correlation with serum N-terminal pro-brain natriuretic peptide concentrations Haematologica 92 738 743
24. LM De Castro JC Jonassaint FL Graham A Ashley-Koch MJ Telen 2008 Pulmonary hypertension associated with sickle cell disease: clinical and laboratory endpoints and disease outcomes Am J Hematol 83 19 25
25. TJ Wang MG Larson D Levy EJ Benjamin EP Leip T Omland PA Wolf RS Vasan 2004 Plasma natriuretic peptide levels and the risk of cardiovascular events and death N Engl J Med 350 655 663
26. C Kragelund B Gronning L Kober P Hildebrandt R Steffensen 2005 N-terminal pro-B-type natriuretic peptide and long-term mortality in stable coronary heart disease N Engl J Med 352 666 675
27. LB Yap H Ashrafian D Mukerjee JG Coghlan PM Timms 2004 The natriuretic peptides and their role in disorders of right heart dysfunction and pulmonary hypertension Clin Biochem 37 847 856
28. RF Machado A Anthi MH Steinberg D Bonds V Sachdev GJ Kato AM Taveira-DaSilva SK Ballas W Blackwelder X Xu L Hunter B Barton M Waclawiw O Castro MT Gladwin, MSH Investigators 2006 N-terminal pro-brain natriuretic peptide levels and risk of death in sickle cell disease JAMA 296 310 318
29. EE Lin MT Gladwin RF Machado 2005 Pulmonary hypertension in patients with hemoglobinopathies: could a mechanism for dysfunction provide an avenue for novel therapeutics? Haematologica 90 441 444
30. N Galie A Manes A Branzi 2004 The endothelin system in pulmonary arterial hypertension Cardiovasc Res 61 227 237
31. ER Levin 1995 Endothelins N Engl J Med 333 356 363
32. AP Davenport 2002 International Union of Pharmacology. XXIX. Update on endothelin receptor nomenclature Pharmacol Rev 54 219 226
33. J Dupuis 2001 Endothelin-receptor antagonists in pulmonary hypertension Lancet 358 1113 1114
34. B Seo BS Oemar R Siebenmann L von Segesser TF Lüscher 1994 Both ETA and ETB receptors mediate contraction to endothelin-1 in human blood vessels Circulation 89 1203 1208
35. P Cacoub R Dorent P Nataf A Carayon 1993 Endothelin-1 in pulmonary hypertension N Engl J Med 329 1967 1968
36. A Giaid M Yanagisawa D Langleben RP Michel R Levy H Shennib S Kimura T Masaki WP Duguid DJ Stewart 1993 Expression of endothelin-1 in the lungs of patients with pulmonary hypertension N Engl J Med 328 1732 1739
37. C Rubens R Ewert M Halank R Wensel HD Orzechowski HP Schultheiss G Hoeffken 2001 Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension Chest 120 1562 1569
38. M Yoshibayashi K Nishioka K Nakao Y Saito M Matsumura T Ueda S Temma G Shirakami H Imura H Mikawa 1991 Plasma endothelin concentrations in patients with pulmonary hypertension associated with congenital heart defects. Evidence for increased production of endothelin in pulmonary circulation Circulation 84 2280 2285
39. E Graido-Gonzalez JC Doherty EW Bergreen G Organ M Telfer MA McMillen 1998 Plasma endothelin-1, cytokine, and prostaglandin E2 levels in sickle cell disease and acute vaso-occlusive sickle crisis Blood 92 2551 2555
40. AC Rybicki LJ Benjamin 1998 Increased levels of endothelin-1 in plasma of sickle cell anemia patients Blood 92 2594 2596
41. YT Shiu LV McIntire MM Udden 2002 Sickle erythrocytes increase prostacyclin and endothelin-1 production by cultured human endothelial cells under flow conditions Eur J Haematol 68 163 169
42. SG Werdehoff RB Moore CJ Hoff E Fillingim AM Hackman 1998 Elevated plasma endothelin-1 levels in sickle cell anemia: relationships to oxygen saturation and left ventricular hypertrophy Am J Hematol 58 195 199
43. A Rivera P Jarolim C Brugnara 2002 Modulation of Gardos channel activity by cytokines in sickle erythrocytes Blood 99 357 603
44. DB Cines ES Pollak CA Buck J Loscalzo GA Zimmerman RP McEver JS Pober TM Wick BA Konkle BS Schwartz ES Barnathan KR McCrae BA Hug AM Schmidt DM Stern 1998 Endothelial cells in physiology and in the pathophysiology of vascular disorders Blood 91 3527 3561
45. G Lin RL Macdonald LS Marton A Kowalczuk NJ Solenski BK Weir 2001 Hemoglobin increases endothelin-1 in endothelial cells by decreasing nitric oxide Biochem Biophys Res Commun 280 824 830
46. SI Hammerman S Kourembanas TJ Conca M Tucci M Brauer HW Farber 1997 Endothelin-1 production during the acute chest syndrome in sickle cell disease Am J Respir Crit Care Med 156 280 285
47. Sabaa N, de Franceschi L, Bonnin P, Malpeli G, Germain S, Vandermeersch S, Maier-Redelsperger M, Scarpa A, Janin A, Levy B, Girot R, Beuzard Y, Leboeuf C, Henri A, Dussaule J-C, Tharaux P-L (2006) The dual endothelin receptor antagonist bosentan prevents the acute sickle cell-related hypoxic lung and kidney injury in transgenic SAD mice. Blood 108:Abstract 687
48. CR Morris 2006 New strategies for the treatment of pulmonary hypertension in sickle cell disease: the rationale for arginine therapy Treat Respir Med 5 31 45
49. CR Morris SM Morris Jr W Hagar J Van Warmerdam S Claster D Kepka-Lenhart L Machado FA Kuypers EP Vichinsky 2003 Arginine therapy: a new treatment for pulmonary hypertension in sickle cell disease? Am J Respir Crit Care Med 168 63 69
50. MT Gladwin AN Schechter 2001 Nitric oxide therapy in sickle cell disease Semin Hematol 38 333 342
51. KJ Sullivan SR Goodwin J Evangelist RD Moore P Mehta 1999 Nitric oxide successfully used to treat acute chest syndrome of sickle cell disease in a young adolescent Crit Care Med 27 2563 2568
52. RF Machado S Martyr GJ Kato RJ Barst A Anthi MR Robinson L Hunter W Coles J Nichols C Hunter V Sachdev O Castro MT Gladwin 2005 Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension Br J Haematol 130 445 453
53. G Derchi GL Forni F Formisano MD Cappellini R Galanello G D'Ascola P Bina C Magnano M Lamagna 2005 Efficacy and safety of sildenafil in the treatment of severe pulmonary hypertension in patients with hemoglobinopathies Haematologica 90 452 458
54. GJ Kato OC Onyekwere MT Gladwin 2007 Pulmonary hypertension in sickle cell disease: relevance to children Pediatr Hematol Oncol 24 159 170
55. DB Badesch SH Abman G Simonneau LJ Rubin VV McLaughlin 2007 Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines Chest 131 1917 1928
56. CD Reiter X Wang JE Tanus-Santos N Hogg RO Cannon 3rd AN Schechter MT Gladwin 2002 Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease Nat Med 8 1383 1389