Prevalence and risk factors for pulmonary arterial hypertension in a large group of β-thalassemia patients using right heart catheterization: A webthal study

Circulation

Volumn 129, Issue 3, 2014, Pages 338-345

  Derchi, Giorgio ^a   Galanello, Renzo ^b   Bina, Patrizio ^b   Cappellini, Maria Domenica ^c   Piga, Antonio ^d   Lai, Maria Eliana ^b   Quarta, Antonella ^e   Casu, Gavino ^f   Perrotta, Silverio ^g   Pinto, Valeria ^a   Musallam, Khaled M ^c   Forni, Gian Luca ^a  

^a GALLIERA HOSPITAL   (Italy)

^b UNIVERSITY OF CAGLIARI   (Italy)

^c UNIVERSITY OF MILAN   (Italy)

^d UNIVERSITY OF TURIN   (Italy)

^e PERRINO HOSPITAL   (Italy)

^f SAN FRANCESCO HOSPITAL   (Italy)

^g SECOND UNIVERSITY OF NAPLES   (Italy)

Author keywords

Catheterization; Echocardiography; Pulmonary arterial hypertension; Risk factors

Indexed keywords

FERRITIN; HEMOGLOBIN;

ADULT; AGE DISTRIBUTION; AGED; ARTICLE; CARDIOVASCULAR RISK; CROSS-SECTIONAL STUDY; FEMALE; FERRITIN BLOOD LEVEL; HEART CATHETERIZATION; HEMOGLOBIN BLOOD LEVEL; HUMAN; LUNG ARTERY PRESSURE; MAJOR CLINICAL STUDY; MALE; MIDDLE AGED; MULTICENTER STUDY; PREDICTIVE VALUE; PREVALENCE; PRIORITY JOURNAL; PULMONARY ARTERY OCCLUSION PRESSURE; PULMONARY HYPERTENSION; SPLENECTOMY; THALASSEMIA INTERMEDIA; THALASSEMIA MAJOR; TRANSTHORACIC ECHOCARDIOGRAPHY; VERY ELDERLY; YOUNG ADULT;

CATHETERIZATION; ECHOCARDIOGRAPHY; PULMONARY ARTERIAL HYPERTENSION; RISK FACTORS;

ADULT; BETA-THALASSEMIA; CARDIAC CATHETERIZATION; CASE-CONTROL STUDIES; CROSS-SECTIONAL STUDIES; ECHOCARDIOGRAPHY; FEMALE; HUMANS; HYPERTENSION, PULMONARY; LOGISTIC MODELS; MALE; MIDDLE AGED; MULTIVARIATE ANALYSIS; PREDICTIVE VALUE OF TESTS; PREVALENCE; PULMONARY WEDGE PRESSURE; RISK FACTORS;

EID: 84893669300     PISSN: 00097322     EISSN: 15244539     Source Type: Journal    
DOI: 10.1161/CIRCULATIONAHA.113.002124     Document Type: Article

References (67)

1. Weatherall DJ. The inherited diseases of hemoglobin are an emerging global health burden. Blood. 2010;115:4331-4336.
2. Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood. 2011;118:3479-3488.
3. Musallam KM, Taher AT, Rachmilewitz EA. ß-thalassemia intermedia: a clinical perspective. Cold Spring Harb Perspect Med. 2012;2:a013482.
4. Danjou F, Anni F, Galanello R. Beta-thalassemia: from genotype to phenotype. Haematologica. 2011;96:1573-1575.
5. Farmakis D, Aessopos A. Pulmonary hypertension associated with hemoglobinopathies: prevalent but overlooked. Circulation. 2011;123: 1227-1232.
6. Morris CR, Vichinsky EP. Pulmonary hypertension in thalassemia. Ann N Y Acad Sci. 2010;1202:205-213.
7. Galie N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2009;34:1219-1263.
8. Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G; ESC Committee for Practice Guidelines (CPG). Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009;30:2493-2537.
9. Badesch DB, Champion HC, Sanchez MA, Hoeper MM, Loyd JE, Manes A, McGoon M, Naeije R, Olschewski H, Oudiz RJ, Torbicki A. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54(1 Suppl):S55-S66.
10. Parent F, Bachir D, Inamo J, Lionnet F, Driss F, Loko G, Habibi A, Bennani S, Savale L, Adnot S, Maitre B, Yaïci A, Hajji L, O'Callaghan DS, Clerson P, Girot R, Galacteros F, Simonneau G. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. 2011;365:44-53.
11. Cappellini MD, Cohen A, Eleftheriou A, Piga A, Porter J, Taher A. Guidelines for the clinical management of thalassemia. Nicosia, Cyprus: Thalassaemia International Federation; 2009.
12. Berger M, Haimowitz A, Van Tosh A, Berdoff RL, Goldberg E. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol. 1985;6:359-365.
13. Kircher BJ, Himelman RB, Schiller NB. Noninvasive estimation of right atrial pressure from the inspiratory collapse of the inferior vena cava. Am J Cardiol. 1990;66:493-496.
14. Lanzarini L, Fontana A, Lucca E, Campana C, Klersy C. Noninvasive estimation of both systolic and diastolic pulmonary artery pressure from Doppler analysis of tricuspid regurgitant velocity spectrum in patients with chronic heart failure. Am Heart J. 2002;144:1087-1094.
15. New York Heart Association. Criteria Committee., New York Heart Association. Nomenclature and criteria for diagnosis of diseases of the heart and great vessels. Boston: Little, Brown; 1994.
16. Taher A, Isma'eel H, Cappellini MD. Thalassemia intermedia: revisited. Blood Cells Mol Dis. 2006;37:12-20.
17. Vlahos AP, Koutsouka FP, Papamichael ND, Makis A, Baltogiannis GG, Athanasiou E, Chaliasos N, Bourantas KL, Kolettis TM. Determinants of pulmonary hypertension in patients with Beta-thalassemia major and normal ventricular function. Acta Haematol. 2012;128:124-129.
18. Chueamuangphan N, Wongtheptien W, Nawarawong W, Sukornthasarn A, Chuncharunee S, Tawichasri C, Patumanond J. Clinical indicators for pulmonary arterial hypertension in thalassemia. J Med Assoc Thai. 2012;95:16-21.
19. Rafsanjani KA, Mafi N, Tafreshi RI. Complications of ß-thalassemia intermedia in Iran during 1996-2010 (single-center study). Pediatr Hematol Oncol. 2011;28:497-508.
20. Amoozgar H, Farhani N, Karimi M. Early echocardiographic findings in ß-thalassemia intermedia patients using standard and tissue Doppler methods. Pediatr Cardiol. 2011;32:154-159.
21. Kiter G, Balci YI, Ates A, Hacioglu S, Sari I. Frequency of pulmonary hypertension in asymptomatic ß-thalassemia major patients and the role of physiological parameters in evaluation. Pediatr Hematol Oncol. 2010;27:597-607.
22. Mokhtar GM, Adly AA, El Alfy MS, Tawfik LM, Khairy AT. N-terminal natriuretic peptide and ventilation-perfusion lung scan in sickle cell disease and thalassemia patients with pulmonary hypertension. Hemoglobin. 2010;34:78-94.
23. El-Beshlawy A, Youssry I, El-Saidi S, El Accaoui R, Mansi Y, Makhlouf A, Taher A. Pulmonary hypertension in beta-thalassemia major and the role of L-carnitine therapy. Pediatr Hematol Oncol. 2008;25:734-743.
24. Phrommintikul A, Sukonthasarn A, Kanjanavanit R, Nawarawong W. Splenectomy: a strong risk factor for pulmonary hypertension in patients with thalassaemia. Heart. 2006;92:1467-1472.
25. Singer ST, Kuypers FA, Styles L, Vichinsky EP, Foote D, Rosenfeld H. Pulmonary hypertension in thalassemia: association with platelet activation and hypercoagulable state. Am J Hematol. 2006;81:670-675.
26. Hagar RW, Morris CR, Vichinsky EP. Pulmonary hypertension in thalassaemia major patients with normal left ventricular systolic function. Br J Haematol. 2006;133:433-435.
27. Aessopos A, Farmakis D, Deftereos S, Tsironi M, Tassiopoulos S, Moyssakis I, Karagiorga M. Thalassemia heart disease: a comparativeevaluation of thalassemia major and thalassemia intermedia. Chest. 2005;127:1523-1530.
28. Aessopos A, Farmakis D, Karagiorga M, Voskaridou E, Loutradi A, Hatziliami A, Joussef J, Rombos J, Loukopoulos D. Cardiac involvement in thalassemia intermedia: a multicenter study. Blood. 2001;97: 3411-3416.
29. Du ZD, Roguin N, Milgram E, Saab K, Koren A. Pulmonary hypertension in patients with thalassemia major. Am Heart J. 1997;134:532-537.
30. Grisaru D, Rachmilewitz EA, Mosseri M, Gotsman M, Lafair JS, Okon E, Goldfarb A, Hasin Y. Cardiopulmonary assessment in beta-thalassemia major. Chest. 1990;98:1138-1142.
31. Isma'eel H, Chafic AH, El Rassi F, Inati A, Koussa S, Daher R, Gharzuddin W, Alam S, Taher A. Relation between iron-overload indices, cardiac echo-Doppler, and biochemical markers in thalassemia intermedia. Am J Cardiol. 2008;102:363-367.
32. Taher AT, Musallam KM, Karimi M, El-Beshlawy A, Belhoul K, Daar S, Saned MS, El-Chafic AH, Fasulo MR, Cappellini MD. Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood. 2010;115:1886-1892.
33. Morris CR, Kim HY, Trachtenberg F, Wood J, Quinn CT, Sweeters N, Kwiatkowski JL, Thompson AA, Giardina PJ, Boudreaux J, Olivieri NF, Porter JB, Neufeld EJ, Vichinsky EP; Thalassemia Clinical Research Network. Risk factors and mortality associated with an elevated tricuspid regurgitant jet velocity measured by Doppler-echocardiography in thalassemia: a Thalassemia Clinical Research Network report. Blood. 2011;118:3794-3802.
34. Atichartakarn V, Likittanasombat K, Chuncharunee S, Chandanamattha P, Worapongpaiboon S, Angchaisuksiri P, Aryurachai K. Pulmonary arterial hypertension in previously splenectomized patients with beta- thalassemicdisorders. Int J Hematol. 2003;78:139-145.
35. Amoozgar H, Farhani N, Karimi M. Risk factors for pulmonary hypertensionin patients with thalassemia intermedia. Eur J Haematol. 2010;85:549-551.
36. Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, Elliott CG, Gaine SP, Gladwin MT, Jing ZC, Krowka MJ, Langleben D, Nakanishi N, Souza R. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54(1 Suppl):S43-S54.
37. Zakynthinos E, Vassilakopoulos T, Kaltsas P, Malagari E, Daniil Z, Roussos C, Zakynthinos SG. Pulmonary hypertension, interstitial lung fibrosis, and lung iron deposition in thalassaemia major. Thorax. 2001;56:737-739.
38. Jootar P, Fucharoen S. Cardiac involvement in beta-thalassemia/hemoglobin E disease: clinical and hemodynamic findings. Southeast Asian J Trop Med Public Health. 1990;21:269-273.
39. Karimi M, Musallam KM, Cappellini MD, Daar S, El-Beshlawy A, Belhoul K, Saned MS, Temraz S, Koussa S, Taher AT. Risk factors for pulmonary hypertension in patients with ß thalassemia intermedia. Eur J Intern Med. 2011;22:607-610.
40. Taher AT, Musallam KM, El-Beshlawy A, Karimi M, Daar S, Belhoul K, Saned MS, Graziadei G, Cappellini MD. Age-related complications in treatment-naïve patients with thalassaemia intermedia. Br J Haematol. 2010;150:486-489.
41. Aessopos A, Farmakis D, Deftereos S, Tsironi M, Polonifi A, Moyssakis I, Diamanti-Kandaraki E, Papalambros E. Cardiovascular effects of splenomegaly and splenectomy in beta-thalassemia. Ann Hematol. 2005;84:353-357.
42. Musallam KM, Taher AT. Thrombosis in thalassemia: why are we so concerned' Hemoglobin. 2011;35:503-510.
43. Meera V, Jijina F, Ghosh K. Pulmonary hypertension in patients with hematological disorders following splenectomy. Indian J Hematol Blood Transfus. 2010;26:2-5.
44. Morris CR, Kuypers FA, Kato GJ, Lavrisha L, Larkin S, Singer T, Vichinsky EP. Hemolysis-associated pulmonary hypertension in thalassemia. Ann N Y Acad Sci. 2005;1054:481-485.
45. Bunn HF, Nathan DG, Dover GJ, Hebbel RP, Platt OS, Rosse WF, Ware RE. Pulmonary hypertension and nitric oxide depletion in sickle cell disease. Blood. 2010;116:687-692.
46. Patel N, Gonsalves CS, Malik P, Kalra VK. Placenta growth factor augments endothelin-1 and endothelin-B receptor expression via hypoxiainducible factor-1 alpha. Blood. 2008;112:856-865.
47. Musallam KM, Cappellini MD, Wood JC, Motta I, Graziadei G, Tamim H, Taher AT. Elevated liver iron concentration is a marker of increased morbidity in patients with ß thalassemia intermedia. Haematologica. 2011;96:1605-1612.
48. Koren A, Garty I, Antonelli D, Katzuni E. Right ventricular cardiac dysfunction in beta-thalassemia major. Am J Dis Child. 1987;141:93-96.
49. Hamdy AM, Zein El-Abdin MY, Abdel-Hafez MA. Right ventricular function in patients with beta thalassemia: relation to serum ferritin level. Echocardiography. 2007;24:795-801.
50. Kell DB. Iron behaving badly: inappropriate iron chelation as a major contributor to the aetiology of vascular and other progressive inflammatory and degenerative diseases. BMC Med Genomics. 2009;2:2.
51. Musallam KM, Taher AT, Karimi M, Rachmilewitz EA. Cerebral infarction in ß-thalassemia intermedia: breaking the silence. Thromb Res. 2012;130:695-702.
52. Musallam KM, Motta I, Salvatori M, Fraquelli M, Marcon A, Taher AT, Cappellini MD. Longitudinal changes in serum ferritin levels correlate with measures of hepatic stiffness in transfusion-independent patients with ß-thalassemia intermedia. Blood Cells Mol Dis. 2012;49:136-139.
53. Derchi G, Lai ME, Marcaccini P, Carta MP, Vacquer S. Human immunodeficiency virus and beta-thalassemia major: A "competition of guilt" for pulmonary arterial hypertension. Report of a case and a review of the literature. Hemoglobin. 2010;34:61-66.
54. Machado RF, Gladwin MT. Pulmonary hypertension in hemolytic disorders: pulmonary vascular disease: the global perspective. Chest. 2010;137 (6 Suppl):30S-38S.
55. Aessopos A, Stamatelos G, Skoumas V, Vassilopoulos G, Mantzourani M, Loukopoulos D. Pulmonary hypertension and right heart failure in patients with beta-thalassemia intermedia. Chest. 1995;107:50-53.
56. Aessopos A, Kati M, Farmakis D. Heart disease in thalassemia intermedia: a review of the underlying pathophysiology. Haematologica. 2007;92:658-665.
57. Kremastinos DT, Farmakis D, Aessopos A, Hahalis G, Hamodraka E, Tsiapras D, Keren A. Beta-thalassemia cardiomyopathy: history, present considerations, and future perspectives. Circ Heart Fail. 2010;3:451-458.
58. Atichartakarn V, Chuncharunee S, Chandanamattha P, Likittanasombat K, Aryurachai K. Correction of hypercoagulability and amelioration ofpulmonary arterial hypertension by chronic blood transfusion in an asplenic hemoglobin E/beta-thalassemia patient. Blood. 2004;103:2844-2846.
59. Musallam KM, Taher AT, Cappellini MD, Sankaran VG. Clinical experiencewith fetal hemoglobin induction therapy in patients with ß-thalassemia. Blood. 2013;121:2199-212; quiz 2372.
60. Amoozgar H, Farhani N, Khodadadi N, Karimi M, Cheriki S. Comparative study of pulmonary circulation and myocardial function in patients with ß-thalassemia intermedia with and without hydroxyurea, a case-control study. Eur J Haematol. 2011;87:61-67.
61. Karimi M, Borzouee M, Mehrabani A, Cohan N. Echocardiographic finding in beta-thalassemia intermedia and major: absence of pulmonary hypertension following hydroxyurea treatment in beta-thalassemia intermedia. Eur J Haematol. 2009;82:213-218.
62. Littera R, La Nasa G, Derchi G, Cappellini MD, Chang CY, Contu L. Long-term treatment with sildenafil in a thalassemic patient with pulmonary hypertension. Blood. 2002;100:1516-1517.
63. Derchi G, Forni GL, Formisano F, Cappellini MD, Galanello R, D'AscolaG, Bina P, Magnano C, Lamagna M. Efficacy and safety of sildenafil in thetreatment of severe pulmonary hypertension in patients with hemoglobinopathies. Haematologica. 2005;90:452-458.
64. Correale M, De Rosa F, Ieva R, Di Biase M, Brunetti ND. Long-term treatment with high-dose of sildenafil in a thalassemic patient with pulmonary hypertension. Monaldi Arch Chest Dis. 2012;78:105-106.
65. Morris CR, Kim H-Y, Wood JC, Trachtenberg F, Klings ES, Porter JB, Sweeters N, Olivieri NF, Kwiatkowski JL, Singer ST, Taher AT, Neufeld EJ, Thompson AA, Sachdev V, Larkin SK, Suh JH, Kuypers FA, Vichinsky E. Sildenafil therapy in patients with thalassemia and an elevated tricuspid regurgitant jet velocity (trv) on doppler echocardiography at risk for pulmonary hypertension: Report from the thalassemia clinical research network [abstract]. Blood. 2012;120:1023.
66. Anthi A, Tsangaris I, Hamodraka ES, Lekakis J, Armaganidis A, Orfanos SE. Treatment with bosentan in a patient with thalassemia intermedia and pulmonary arterial hypertension. Blood. 2012;120:1531-1532.
67. Tam DH, Farber HW. Pulmonary hypertension and beta-thalassemia major: report of a case, its treatment, and a review of the literature. Am J Hematol. 2006;81:443-447.