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Published in: Annals of Hematology 11/2016

01-11-2016 | Review Article

The global burden of pulmonary hypertension in sickle cell disease: a systematic review and meta-analysis

Authors: B. M. Musa, N. A. Galadanci, M. Coker, S. Bussell, M. H. Aliyu

Published in: Annals of Hematology | Issue 11/2016

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Abstract

Elevated tricuspid regurgitant jet velocity (TRJV) is a surrogate measure of pulmonary hypertension (PH) in persons with sickle cell disease (SCD). We sought to estimate the burden of PH in people living with sickle cell disease based on TRJV. From 2000 to 2015, we searched electronic databases for eligible publications and included 29 studies (n = 5358 persons). We used random effects modeling to determine the pooled estimate of elevated TRJV. The overall pooled prevalence of elevated TRJV was 23.5 %(95 % CI 19.5–27.4) in persons with SCD. The pooled prevalence of elevated TRJV in children and adults with SCD was 20.7 % (95 % CI 15.7–-25.6) and 24.4 % (95 % CI 18.4–30.4), respectively. TRJV is prevalent among adults and children with SCD. Our finding support international recommendations that call for screening for PH in SCD patients.
Literature
1.
go back to reference Aessopos A, Farmakis D, Karagiorga M et al (2001) Cardiac involvement in thalassemia intermedia: a multicenter study. Blood 97:3411–3416CrossRefPubMed Aessopos A, Farmakis D, Karagiorga M et al (2001) Cardiac involvement in thalassemia intermedia: a multicenter study. Blood 97:3411–3416CrossRefPubMed
3.
go back to reference Yater W, Hansmann G (1936) Sickle cell anemia: a new cause of cor pulmonale: report of two cases with numerous disseminated occlusions of the small pulmonary arteries. Am J Med Sci 191:474–484CrossRef Yater W, Hansmann G (1936) Sickle cell anemia: a new cause of cor pulmonale: report of two cases with numerous disseminated occlusions of the small pulmonary arteries. Am J Med Sci 191:474–484CrossRef
5.
go back to reference Haque AK, Gokhale S, Rampy BA et al (2002) Pulmonary hypertension in sickle cell hemoglobinopathy: a clinicopathologic study of 20 cases. Hum Pathol 33:1037–1043CrossRefPubMed Haque AK, Gokhale S, Rampy BA et al (2002) Pulmonary hypertension in sickle cell hemoglobinopathy: a clinicopathologic study of 20 cases. Hum Pathol 33:1037–1043CrossRefPubMed
7.
8.
go back to reference Powars D, Weidman JA, Odom-Maryon T et al (1988) Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine (Baltimore) 67:66–76CrossRef Powars D, Weidman JA, Odom-Maryon T et al (1988) Sickle cell chronic lung disease: prior morbidity and the risk of pulmonary failure. Medicine (Baltimore) 67:66–76CrossRef
11.
go back to reference Simmons BE, Santhanam V, Castaner A et al (1988) Sickle cell heart disease. Two-dimensional echo and Doppler ultrasonographic findings in the hearts of adult patients with sickle cell anemia. Arch Intern Med 148:1526–1528CrossRefPubMed Simmons BE, Santhanam V, Castaner A et al (1988) Sickle cell heart disease. Two-dimensional echo and Doppler ultrasonographic findings in the hearts of adult patients with sickle cell anemia. Arch Intern Med 148:1526–1528CrossRefPubMed
16.
go back to reference Suell MN, Bezold LI, Okcu MF et al (2005) Increased pulmonary artery pressures among adolescents with sickle cell disease. J Pediatr Hematol Oncol 27:654–658CrossRefPubMed Suell MN, Bezold LI, Okcu MF et al (2005) Increased pulmonary artery pressures among adolescents with sickle cell disease. J Pediatr Hematol Oncol 27:654–658CrossRefPubMed
17.
go back to reference Ambrusko SJ, Gunawardena S, Sakara A et al (2006) Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease. Pediatr Blood Cancer 47:907–913. doi:10.1002/pbc.20791 CrossRefPubMed Ambrusko SJ, Gunawardena S, Sakara A et al (2006) Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease. Pediatr Blood Cancer 47:907–913. doi:10.​1002/​pbc.​20791 CrossRefPubMed
20.
go back to reference Cabrita IZ, Mohammed A, Layton M et al (2013) The association between tricuspid regurgitation velocity and 5-year survival in a North West London population of patients with sickle cell disease in the United Kingdom. Br J Haematol 162:400–408. doi:10.1111/bjh.12391 CrossRefPubMed Cabrita IZ, Mohammed A, Layton M et al (2013) The association between tricuspid regurgitation velocity and 5-year survival in a North West London population of patients with sickle cell disease in the United Kingdom. Br J Haematol 162:400–408. doi:10.​1111/​bjh.​12391 CrossRefPubMed
21.
go back to reference Enakpene EO, Adebiyi AA, Ogah OS et al (2014) Non-invasive estimation of pulmonary artery pressures in patients with sickle cell anaemia in Ibadan, Nigeria: an echocardiographic study. Acta Cardiol 69:505–511PubMed Enakpene EO, Adebiyi AA, Ogah OS et al (2014) Non-invasive estimation of pulmonary artery pressures in patients with sickle cell anaemia in Ibadan, Nigeria: an echocardiographic study. Acta Cardiol 69:505–511PubMed
22.
go back to reference Soh MC, Sankaran S, Nelson-Piercy C, Oteng-Ntim E (2014) The prevalence of raised tricuspid regurgitation velocity in pregnant women with sickle cell disease and its association with obstetric outcomes-an observational study. Societies of Obstetric Medicine combined 2014 meeting in New Orleans, Louisiana, USA: Obstetric Medicine: The Medicine of Pregnancy 8:28–50. doi:10.1177/1753495X14566755 Soh MC, Sankaran S, Nelson-Piercy C, Oteng-Ntim E (2014) The prevalence of raised tricuspid regurgitation velocity in pregnant women with sickle cell disease and its association with obstetric outcomes-an observational study. Societies of Obstetric Medicine combined 2014 meeting in New Orleans, Louisiana, USA: Obstetric Medicine: The Medicine of Pregnancy 8:28–50. doi:10.​1177/​1753495X14566755​
23.
26.
go back to reference Liem RI, Nevin MA, Prestridge A et al (2009) Tricuspid regurgitant jet velocity elevation and its relationship to lung function in pediatric sickle cell disease. Pediatr Pulmonol 44:281–289. doi:10.1002/ppul.20996 CrossRefPubMed Liem RI, Nevin MA, Prestridge A et al (2009) Tricuspid regurgitant jet velocity elevation and its relationship to lung function in pediatric sickle cell disease. Pediatr Pulmonol 44:281–289. doi:10.​1002/​ppul.​20996 CrossRefPubMed
30.
31.
go back to reference Sachdev V, Kato GJ, Gibbs JSR et al (2011) 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 124:1452–1460. doi:10.1161/CIRCULATIONAHA.111.032920 CrossRefPubMedPubMedCentral Sachdev V, Kato GJ, Gibbs JSR et al (2011) 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 124:1452–1460. doi:10.​1161/​CIRCULATIONAHA.​111.​032920 CrossRefPubMedPubMedCentral
33.
36.
37.
go back to reference Upadhya B, Stacey RB, Ntim W et al (2014) Echocardiography-derived tricuspid regurgitant jet velocity is an important marker for the progression of sickle-cell disease. Acta Haematol 132:152–158. doi:10.1159/000357393 CrossRefPubMed Upadhya B, Stacey RB, Ntim W et al (2014) Echocardiography-derived tricuspid regurgitant jet velocity is an important marker for the progression of sickle-cell disease. Acta Haematol 132:152–158. doi:10.​1159/​000357393 CrossRefPubMed
38.
go back to reference Agha H, El Tagui M, El Ghamrawy M, Abdel Hady M (2014) The 6-min walk test: an independent correlate of elevated tricuspid regurgitant jet velocity in children and young adult sickle cell patients. Ann Hematol 93:1131–1138. doi:10.1007/s00277-014-2030-3 PubMed Agha H, El Tagui M, El Ghamrawy M, Abdel Hady M (2014) The 6-min walk test: an independent correlate of elevated tricuspid regurgitant jet velocity in children and young adult sickle cell patients. Ann Hematol 93:1131–1138. doi:10.​1007/​s00277-014-2030-3 PubMed
39.
go back to reference Molavi MA, Rajaei S, Elahi Z, et al (2015) Evaluation of tricuspid regurgitation jet velocity in children with sickle cell disease in Iran 2012-2013. Advances in Bioresearch 6(5);211 Molavi MA, Rajaei S, Elahi Z, et al (2015) Evaluation of tricuspid regurgitation jet velocity in children with sickle cell disease in Iran 2012-2013. Advances in Bioresearch 6(5);211
40.
go back to reference Mushemi-Blake S, Melikian N, Drasar E, et al (2015) Pulmonary haemodynamics in sickle cell disease are driven predominantly by a high-output state rather than elevated pulmonary vascular resistance: a prospective 3-dimensional echocardiography/doppler study. PLoS One. doi: 10.1371/journal.pone.0135472 Mushemi-Blake S, Melikian N, Drasar E, et al (2015) Pulmonary haemodynamics in sickle cell disease are driven predominantly by a high-output state rather than elevated pulmonary vascular resistance: a prospective 3-dimensional echocardiography/doppler study. PLoS One. doi: 10.​1371/​journal.​pone.​0135472
41.
go back to reference Hebson C, New T, Record E et al (2015) Elevated tricuspid regurgitant velocity as a marker for pulmonary hypertension in children with sickle cell disease: less prevalent and predictive than previously thought? J Pediatr Hematol Oncol 37:134–139. doi:10.1097/MPH.0000000000000184 CrossRefPubMed Hebson C, New T, Record E et al (2015) Elevated tricuspid regurgitant velocity as a marker for pulmonary hypertension in children with sickle cell disease: less prevalent and predictive than previously thought? J Pediatr Hematol Oncol 37:134–139. doi:10.​1097/​MPH.​0000000000000184​ CrossRefPubMed
42.
45.
go back to reference Stroup DF, Berlin JA, Morton SC et al (2000) Meta-analysis of observational studies in epidemiology: a proposal for reporting. Meta-analysis Of Observational Studies in Epidemiology (MOOSE) group. JAMA 283:2008–2012CrossRefPubMed Stroup DF, Berlin JA, Morton SC et al (2000) Meta-analysis of observational studies in epidemiology: a proposal for reporting. Meta-analysis Of Observational Studies in Epidemiology (MOOSE) group. JAMA 283:2008–2012CrossRefPubMed
46.
go back to reference Begg CB, Mazumdar M (1994) Operating characteristics of a rank correlation test for publication bias. Biometrics 50:1088–1101CrossRefPubMed Begg CB, Mazumdar M (1994) Operating characteristics of a rank correlation test for publication bias. Biometrics 50:1088–1101CrossRefPubMed
48.
go back to reference Sutton LL, Castro O, Cross DJ et al (1994) Pulmonary hypertension in sickle cell disease. Am J Cardiol 74:626–628CrossRefPubMed Sutton LL, Castro O, Cross DJ et al (1994) Pulmonary hypertension in sickle cell disease. Am J Cardiol 74:626–628CrossRefPubMed
49.
go back to reference Castro O (1996) Systemic fat embolism and pulmonary hypertension in sickle cell disease. Hematol Oncol Clin North Am 10:1289–1303CrossRefPubMed Castro O (1996) Systemic fat embolism and pulmonary hypertension in sickle cell disease. Hematol Oncol Clin North Am 10:1289–1303CrossRefPubMed
56.
go back to reference Machado R, Barst R, Yovetich N, et al (2009) Safety and efficacy of sildenafil therapy for Doppler-defined pulmonary hypertension in patients with sickle cell disease: preliminary results of the Walk- PHaSST clinical trial. American Society of Hematology. In: Top 10 clinically oriented abstracts in sickle cell disease. American Journal of Hematology, n New Orleans, Louisiana, USA, pp 204–205 Machado R, Barst R, Yovetich N, et al (2009) Safety and efficacy of sildenafil therapy for Doppler-defined pulmonary hypertension in patients with sickle cell disease: preliminary results of the Walk- PHaSST clinical trial. American Society of Hematology. In: Top 10 clinically oriented abstracts in sickle cell disease. American Journal of Hematology, n New Orleans, Louisiana, USA, pp 204–205
Metadata
Title
The global burden of pulmonary hypertension in sickle cell disease: a systematic review and meta-analysis
Authors
B. M. Musa
N. A. Galadanci
M. Coker
S. Bussell
M. H. Aliyu
Publication date
01-11-2016
Publisher
Springer Berlin Heidelberg
Published in
Annals of Hematology / Issue 11/2016
Print ISSN: 0939-5555
Electronic ISSN: 1432-0584
DOI
https://doi.org/10.1007/s00277-016-2693-z

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