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Subclinical left ventricular systolic impairment in steady state young adult patients with sickle-cell anemia

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Abstract

Chronic volume overload in sickle-cell anemia (SCA) is associated with left ventricular (LV) enlargement and hypertrophy. The effect of the disease on LV systolic function remains debated. The aim of our study was to investigate LV systolic function in SCA patients using 2D speckle-tracking imaging. We compared 30 steady state asymptomatic adult SCA patients (17 women, mean age 24.7 ± 5.1 years) with 30 age and sex-matched healthy subjects (17 women, mean age 25.0 ± 4.9 years). In addition to conventional echocardiographic parameters including LV ejection fraction (LVEF) and LV mass index (LVMi), global longitudinal strain (GLS) and strain rate (GLSR) were measured. GLS (−17.9 ± 2.0 vs. −19.7 ± 2.5 %, p = 0.004) and GLSR (−0.92 ± 0.09 vs. −1.07 ± 0.17 s−1, p < 0.0001) values were lower in SCA patients while LVEF values (60.1 ± 3.8 vs. 61.7 ± 4.7 %, p = 0.30) were not different. LVMi was increased in SCA patients (100.7 ± 23.5 vs. 72.4 ± 15.2 g/m2, p = 0.0001) and GLSR was significantly lower in the subgroup of patients with LV hypertrophy (−0.88 ± 0.09 vs. −0.96 ± 0.08 s−1, p = 0.02). In SCA patients LVMi was correlated to GLS (r = 0.58, p = 0.001) and GLSR (r = 0.45, p = 0.015) pleading in favor of a pathological LV remodeling. Asymptomatic SCA patients exhibited a subclinical alteration of LV systolic function. Myocardial dysfunction appears to be linked to the degree of LV hypertrophy. 2D speckle-tracking imaging might be useful for long-term follow-up and to study the natural course of LV dysfunction in SCA patients.

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References

  1. Weatherall D, Hofman K, Rodgers G et al (2005) A case for developing north–south partnerships for research in sickle cell disease. Blood 105:921–923. doi:10.1182/blood-2004-06-2404

    Article  PubMed  CAS  Google Scholar 

  2. Rees DC, Williams TN, Gladwin MT (2010) Sickle-cell disease. Lancet 376:2018–2031. doi:10.1016/S0140-6736(10)61029-X

    Article  PubMed  CAS  Google Scholar 

  3. Fitzhugh CD, Lauder N, Jonassaint JC et al (2010) Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease. Am J Hematol 85:36–40. doi:10.1002/ajh.21569

    PubMed  Google Scholar 

  4. Gladwin MT, Sachdev V (2012) Cardiovascular abnormalities in sickle cell disease. J Am Coll Cardiol 59:1123–1133. doi:10.1016/j.jacc.2011.10.900

    Article  PubMed  Google Scholar 

  5. Batra AS, Acherman RJ, Wong W et al (2002) Cardiac abnormalities in children with sickle cell anemia. Am J Hematol 70:306–312. doi:10.1002/ajh.10154

    Article  PubMed  Google Scholar 

  6. Lester LA, Sodt PC, Hutcheon N, Arcilla RA (1990) Cardiac abnormalities in children with sickle cell anemia. Chest 98:1169–1174

    Article  PubMed  CAS  Google Scholar 

  7. Vahanian A, Alfieri O, Andreotti F et al (2012) Guidelines on the management of valvular heart disease (version 2012): the Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS). Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg 42:S1–S44. doi:10.1093/ejcts/ezs455

    Article  Google Scholar 

  8. Naito Y, Tsujino T, Matsumoto M et al (2009) Adaptive response of the heart to long-term anemia induced by iron deficiency. Am J Physiol Heart Circ Physiol 296:H585–H593. doi:10.1152/ajpheart.00463.2008

    Article  PubMed  CAS  Google Scholar 

  9. Voskaridou E, Christoulas D, Terpos E (2012) Sickle-cell disease and the heart: review of the current literature. Br J Haematol 157:664–673. doi:10.1111/j.1365-2141.2012.09143.x

    Article  PubMed  CAS  Google Scholar 

  10. Eddine AC, Alvarez O, Lipshultz SE et al (2012) Ventricular structure and function in children with sickle cell disease using conventional and tissue Doppler echocardiography. Am J Cardiol 109:1358–1364. doi:10.1016/j.amjcard.2012.01.001

    Article  PubMed  Google Scholar 

  11. Lamers L, Ensing G, Pignatelli R et al (2006) Evaluation of left ventricular systolic function in pediatric sickle cell anemia patients using the end-systolic wall stress-velocity of circumferential fiber shortening relationship. J Am Coll Cardiol 47:2283–2288. doi:10.1016/j.jacc.2006.03.005

    Article  PubMed  Google Scholar 

  12. Covitz W, Espeland M, Gallagher D et al (1995) The heart in sickle cell anemia. The Cooperative Study of Sickle Cell Disease (CSSCD). Chest 108:1214–1219

    Article  PubMed  CAS  Google Scholar 

  13. Mor-Avi V, Lang RM, Badano LP et al (2011) Current and evolving echocardiographic techniques for the quantitative evaluation of cardiac mechanics: ASE/EAE consensus statement on methodology and indications endorsed by the Japanese Society of Echocardiography. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr 24:277–313. doi:10.1016/j.echo.2011.01.015

    Article  Google Scholar 

  14. Quiñones MA, Otto CM, Stoddard M et al (2002) Recommendations for quantification of Doppler echocardiography: a report from the Doppler Quantification Task Force of the Nomenclature and Standards Committee of the American Society of Echocardiography. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr 15:167–184

    Article  Google Scholar 

  15. Lang RM, Bierig M, Devereux RB et al (2005) Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr 18:1440–1463. doi:10.1016/j.echo.2005.10.005

    Article  Google Scholar 

  16. Nagueh SF, Appleton CP, Gillebert TC et al (2009) Recommendations for the evaluation of left ventricular diastolic function by echocardiography. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr 22:107–133. doi:10.1016/j.echo.2008.11.023

    Article  Google Scholar 

  17. Sachdev V, Machado RF, Shizukuda Y et al (2007) Diastolic dysfunction is an independent risk factor for death in patients with sickle cell disease. J Am Coll Cardiol 49:472–479. doi:10.1016/j.jacc.2006.09.038

    Article  PubMed  PubMed Central  Google Scholar 

  18. Parent F, Bachir D, Inamo J et al (2011) A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med 365:44–53. doi:10.1056/NEJMoa1005565

    Article  PubMed  CAS  Google Scholar 

  19. Sun K, Xia Y (2013) New insights into sickle cell disease: a disease of hypoxia. Curr Opin Hematol 20:215–221. doi:10.1097/MOH.0b013e32835f55f9

    Article  PubMed  CAS  Google Scholar 

  20. Denenberg BS, Criner G, Jones R, Spann JF (1983) Cardiac function in sickle cell anemia. Am J Cardiol 51:1674–1678

    Article  PubMed  CAS  Google Scholar 

  21. Varat MA, Adolph RJ, Fowler NO (1972) Cardiovascular effects of anemia. Am Heart J 83:415–426

    Article  PubMed  CAS  Google Scholar 

  22. Takahashi M, Kurokawa S, Tsuyusaki T, Kikawada R (1990) Studies of hyperkinetic circulatory state in chronic anemia. J Cardiol 20:331–339

    PubMed  CAS  Google Scholar 

  23. Grossman W, Jones D, McLaurin LP (1975) Wall stress and patterns of hypertrophy in the human left ventricle. J Clin Invest 56:56–64. doi:10.1172/JCI108079

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  24. Balfour IC, Covitz W, Davis H et al (1984) Cardiac size and function in children with sickle cell anemia. Am Heart J 108:345–350

    Article  PubMed  CAS  Google Scholar 

  25. Covarrubias EA, Sheikh MU, Solanki DL et al (1980) Left ventricular function in sickle cell anemia: a noninvasive evaluation. South Med J 73:342–344

    Article  PubMed  CAS  Google Scholar 

  26. Gerry JL, Baird MG, Fortuin NJ (1976) Evaluation of left ventricular function in patients with sickle cell anemia. Am J Med 60:968–972

    Article  PubMed  CAS  Google Scholar 

  27. Sutherland GR, Di Salvo G, Claus P et al (2004) Strain and strain rate imaging: a new clinical approach to quantifying regional myocardial function. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr 17:788–802. doi:10.1016/j.echo.2004.03.027

    Article  Google Scholar 

  28. Burns AT, La Gerche A, D’hooge J et al (2010) Left ventricular strain and strain rate: characterization of the effect of load in human subjects. Eur J Echocardiogr J Work Group Echocardiogr Eur Soc Cardiol 11:283–289. doi:10.1093/ejechocard/jep214

    Article  Google Scholar 

  29. Rösner A, Bijnens B, Hansen M et al (2009) Left ventricular size determines tissue Doppler-derived longitudinal strain and strain rate. Eur J Echocardiogr J Work Group Echocardiogr Eur Soc Cardiol 10:271–277. doi:10.1093/ejechocard/jen230

    Article  Google Scholar 

  30. Poludasu S, Ramkissoon K, Salciccioli L et al (2013) Left ventricular systolic function in sickle cell anemia: a meta-analysis. J Card Fail 19:333–341. doi:10.1016/j.cardfail.2013.03.009

    Article  PubMed  Google Scholar 

  31. Pelliccia A, Maron MS, Maron BJ (2012) Assessment of left ventricular hypertrophy in a trained athlete: differential diagnosis of physiologic athlete’s heart from pathologic hypertrophy. Prog Cardiovasc Dis 54:387–396. doi:10.1016/j.pcad.2012.01.003

    Article  PubMed  Google Scholar 

  32. Galderisi M, Lomoriello VS, Santoro A et al (2010) Differences of myocardial systolic deformation and correlates of diastolic function in competitive rowers and young hypertensives: a speckle-tracking echocardiography study. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr 23:1190–1198. doi:10.1016/j.echo.2010.07.010

    Article  Google Scholar 

  33. Baggish AL, Yared K, Wang F et al (2008) The impact of endurance exercise training on left ventricular systolic mechanics. Am J Physiol Heart Circ Physiol 295:H1109–H1116. doi:10.1152/ajpheart.00395.2008

    Article  PubMed  CAS  Google Scholar 

  34. Popović ZB, Kwon DH, Mishra M et al (2008) Association between regional ventricular function and myocardial fibrosis in hypertrophic cardiomyopathy assessed by speckle tracking echocardiography and delayed hyperenhancement magnetic resonance imaging. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr 21:1299–1305. doi:10.1016/j.echo.2008.09.011

    Article  Google Scholar 

  35. Blanc J, Stos B, de Montalembert M et al (2012) Right ventricular systolic strain is altered in children with sickle cell disease. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr 25:511–517. doi:10.1016/j.echo.2012.01.011

    Article  Google Scholar 

  36. Sengupta SP, Jaju R, Nugurwar A et al (2012) Left ventricular myocardial performance assessed by 2-dimensional speckle tracking echocardiography in patients with sickle cell crisis. Indian Heart J 64:553–558. doi:10.1016/j.ihj.2012.07.021

    Article  PubMed  PubMed Central  Google Scholar 

  37. Ahmad H, Gayat E, Yodwut C et al (2012) Evaluation of myocardial deformation in patients with sickle cell disease and preserved ejection fraction using three-dimensional speckle tracking echocardiography. Echocardiography 29:962–969. doi:10.1111/j.1540-8175.2012.01710.x

    Article  PubMed  PubMed Central  Google Scholar 

  38. Lionnet F, Hammoudi N, Stojanovic KS et al (2012) Hemoglobin sickle cell disease complications: a clinical study of 179 cases. Haematologica 97:1136–1141. doi:10.3324/haematol.2011.055202

    Article  PubMed  PubMed Central  Google Scholar 

  39. Caldas MC, Meira ZA, Barbosa MM (2008) Evaluation of 107 patients with sickle cell anemia through tissue Doppler and myocardial performance index. J Am Soc Echocardiogr Off Publ Am Soc Echocardiogr 21:1163–1167. doi:10.1016/j.echo.2007.06.001

    Article  Google Scholar 

  40. Johnson MC, Kirkham FJ, Redline S et al (2010) Left ventricular hypertrophy and diastolic dysfunction in children with sickle cell disease are related to asleep and waking oxygen desaturation. Blood 116:16–21. doi:10.1182/blood-2009-06-227447

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  41. Feigenbaum H, Mastouri R, Sawada S (2012) A practical approach to using strain echocardiography to evaluate the left ventricle. Circ J Off J Jpn Circ Soc 76:1550–1555

    Google Scholar 

  42. Kalogeropoulos AP, Georgiopoulou VV, Gheorghiade M, Butler J (2012) Echocardiographic evaluation of left ventricular structure and function: new modalities and potential applications in clinical trials. J Card Fail 18:159–172. doi:10.1016/j.cardfail.2011.10.019

    Article  PubMed  Google Scholar 

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Hammoudi, N., Arangalage, D., Djebbar, M. et al. Subclinical left ventricular systolic impairment in steady state young adult patients with sickle-cell anemia. Int J Cardiovasc Imaging 30, 1297–1304 (2014). https://doi.org/10.1007/s10554-014-0473-1

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