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Impact of sickle cell anaemia on cardiac chamber size in the paediatric population

Published online by Cambridge University Press:  14 November 2016

Philippe M. Adjagba ,
Gaston Habib ,
Nancy Robitaille ,
Yves Pastore ,
Marie-Josée Raboisson ,
Daniel Curnier  and
Nagib Dahdah
Philippe M. Adjagba
Affiliation:
Division of Paediatric Cardiology, Centre Hospitalier et Universitaire Ste-Justine, University of Montreal, Montréal, Canada Department of Cardiology, Centre Hospitalier et Universitaire de la Mère et de l’Enfant-Lagune, Cotonou, Bénin
Gaston Habib
Affiliation:
Division of Paediatric Cardiology, Centre Hospitalier et Universitaire Ste-Justine, University of Montreal, Montréal, Canada
Nancy Robitaille
Affiliation:
Department of Pediatrics, Division of Pediatric Haematology-Oncology, Centre Hospitalier et Universitaire Ste-Justine, Montréal, Canada
Yves Pastore
Affiliation:
Department of Pediatrics, Division of Pediatric Haematology-Oncology, Centre Hospitalier et Universitaire Ste-Justine, Montréal, Canada
Marie-Josée Raboisson
Affiliation:
Division of Paediatric Cardiology, Centre Hospitalier et Universitaire Ste-Justine, University of Montreal, Montréal, Canada
Daniel Curnier
Affiliation:
Department of Kinesiology, University of Montreal, Montréal, Canada
Nagib Dahdah*
Affiliation:
Division of Paediatric Cardiology, Centre Hospitalier et Universitaire Ste-Justine, University of Montreal, Montréal, Canada
*
Correspondence to: N. Dahdah, MD, FACC, FASE, FRCPC, Division of Paediatric Cardiology, Centre Hospitalier et Universitaire Sainte-Justine, 3175, Côte Sainte-Catherine, Montréal, Québec, Canada, H3T 1C5. Tel: +514 345 4931, ext 5403; Fax: +514 345 4896; E-mail: nagib.dahdah.hsj@ssss.gouv.qc.ca
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Abstract

Purpose

Sickle cell disease is known to cause various degrees of vasculopathy, including impact on heart function. The aims of this single-centre, retrospective study were to assess cardiac chamber size and function and the relationship with haematological indices such as haemoglobin, aspartate aminotransferase, reticulocytosis and bilirubin, lactate dehydrogenase in sickle cell disease.

Methods

Right ventricle and left ventricle diastolic diameters, left ventricle mass estimate, left ventricle shortening fraction, myocardial performance index, and an index of myocardial relaxation (E/E’) were calculated and correlated with haematological parameters.

Results

A total of 110 patients (65% haemoglobin SS, 29% haemoglobin SC) were studied at a mean age of 12.14±5.26 years. Right ventricle dilatation and left ventricle dilatation were present in 61.5 and 42.9%, respectively. Left ventricle mass was abnormal in 21.9%; all patients had normal myocardial performance index, 31.4% had abnormal E/E’, and left ventricle shortening fraction was low in 38.1%. Cardiac dilatation was best correlated with haemoglobin, aspartate aminotransferase, reticulocytosis and bilirubin. Best subset regression analysis yielded significant additional prediction for right ventricle or left ventricle dilatation with haemoglobin, bilirubin, and lactate dehydrogenase. Abnormal E/E’ was solely predictable with haemoglobin level. Hydroxyurea-treated patients had improved diastolic function.

Conclusion

Right ventricle dilatation was more prevalent than left ventricle dilatation. The long-term consequences of right ventricular dilatation, clinical consequences, and association with pulmonary vasculopathy need to be further determined.

Keywords

Sickle cell anaemiadrepanocytosisright ventriclechild
Type
Original Articles
Information
Cardiology in the Young , Volume 27 , Issue 5 , July 2017 , pp. 918 - 924
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Copyright
© Cambridge University Press 2016 

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References

1. James, TN. Homage to James B. Herrick: a contemporary look at myocardial infarction and at sickle-cell heart disease: the 32nd Annual Herrick Lecture of the Council on Clinical Cardiology of the American Heart Association. Circulation 2000; 101: 1874–1887.Google Scholar
2. Pearson, HA. Sickle cell diseases: diagnosis and management in infancy and childhood. Pediatr Rev 1987; 9: 121–130.Google Scholar
3. Driscoll, MC. Sickle cell disease. Pediatr Rev 2007; 28: 259–268.Google Scholar
4. Denenberg, BS, Criner, G, Jones, R, Spann, JF. Cardiac function in sickle cell anemia. Am J Cardiol 1983; 51: 1674–1678.Google Scholar
5. Covitz, W, Espeland, M, Gallagher, D, Hellenbrand, W, Leff, S, Talner, N. The heart in sickle cell anemia. The Cooperative Study of Sickle Cell Disease (CSSCD). Chest 1995; 108: 1214–1219.Google Scholar
6. Pannu, R, Zhang, J, Andraws, R, Armani, A, Patel, P, Mancusi-Ungaro, P. Acute myocardial infarction in sickle cell disease: a systematic review. Crit Pathw Cardiol 2008; 7: 133–138.CrossRefGoogle ScholarPubMed
7. Hankins, JS, McCarville, MB, Hillenbrand, CM, et al. Ventricular diastolic dysfunction in sickle cell anemia is common but not associated with myocardial iron deposition. Pediatr Blood Cancer 2010; 55: 495–500.Google Scholar
8. Naoman, SG, Nouraie, M, Castro, OL, et al. Echocardiographic findings in patients with sickle cell disease. Ann Hematol 2010; 89: 61–66.Google Scholar
9. Kampmann, C, Wiethoff, C, Wenzel, A, et al. Normal values of M mode echocardiographic measurements of more than 2000 healthy infants and children in central Europe. Heart 2000; 83: 667–672.Google Scholar
10. Foster, BJ, Mackie, AS, Mitsnefes, M, Ali, H, Mamber, S, Colan, SD. A novel method of expressing left ventricular mass relative to body size in children. Circulation 2008; 117: 2769–2775.Google Scholar
11. Tei, C, Ling, LH, Hodge, DO, et al. New index of combined systolic and diastolic myocardial performance: a simple and reproducible measure of cardiac function – a study in normals and dilated cardiomyopathy. J Cardiol 1995; 26: 357–366.Google Scholar
12. Mori, K, Hayabuchi, Y, Kuroda, Y, Nii, M, Manabe, T. Left ventricular wall motion velocities in healthy children measured by pulsed wave Doppler tissue echocardiography: normal values and relation to age and heart rate. J Am Soc Echocardiogr 2000; 13: 1002–1011.Google Scholar
13. Ghaderian, M, Keikhaei, B, Heidari, M, Salehi, Z, Azizi Malamiri, R. Tissue Doppler echocardiographic findings of left ventricle in children with sickle-cell anemia. J Tehran Heart Cent 2012; 7: 106–110.Google Scholar
14. Abdul-Mohsen, MF. Echocardiographic evaluation of left ventricular diastolic and systolic function in Saudi patients with sickle cell disease. J Saudi Heart Assoc 2012; 24: 217–224.Google Scholar
15. Zilberman, MV, Du, W, Das, S, Sarnaik, SA. Evaluation of left ventricular diastolic function in pediatric sickle cell disease patients. Am J Hematol 2007; 82: 433–438.Google Scholar
16. Lamers, L, Ensing, G, Pignatelli, R, et al. 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 2006; 47: 2283–2288.Google Scholar
17. Sachdev, V, Machado, RF, Shizukuda, Y, et al. Diastolic dysfunction is an independent risk factor for death in patients with sickle cell disease. J Am Coll Cardiol 2007; 49: 472–479.Google Scholar
18. Lester, LA, Sodt, PC, Hutcheon, N, Arcilla, RA. Cardiac abnormalities in children with sickle cell anemia. Chest 1990; 98: 1169–1174.Google Scholar
19. Varat, MA, Adolph, RJ, Fowler, NO. Cardiovascular effects of anemia. Am Heart J 1972; 83: 415–426.CrossRefGoogle ScholarPubMed
20. Gerry, JL, Baird, MG, Fortuin, NJ. Evaluation of left ventricular function in patients with sickle cell anemia. Am J Med 1976; 60: 968–972.Google Scholar
21. Batra, AS, Acherman, RJ, Wong, WY, et al. Cardiac abnormalities in children with sickle cell anemia. Am J Hematol 2002; 70: 306–312.Google Scholar
22. de Montalembert, M, Maunoury, C, Acar, P, Brousse, V, Sidi, D, Lenoir, G. Myocardial ischaemia in children with sickle cell disease. Arch Dis Child 2004; 89: 359–362.Google Scholar
23. Gladwin, MT, Sachdev, V. Cardiovascular abnormalities in sickle cell disease. J Am Coll Cardiol 2012; 59: 1123–1133.Google Scholar
24. Gladwin, MT, Barst, RJ, Castro, OL, et al. Pulmonary hypertension and NO in sickle cell. Blood 2010; 116: 852–854.Google Scholar
25. Doherty, DH, Doyle, MP, Curry, SR, et al. Rate of reaction with nitric oxide determines the hypertensive effect of cell-free hemoglobin. Nat Biotechnol 1998; 16: 672–676.Google Scholar
26. Machado, RF, Barst, RJ, Yovetich, NA, et al. Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity. Blood 2011; 118: 855–864.Google Scholar
27. Parent, F, Bachir, D, Inamo, J, et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med 2011; 365: 44–53.Google Scholar
28. Gladwin, MT, Vichinsky, E. Pulmonary complications of sickle cell disease. N Engl J Med 2008; 359: 2254–2265.CrossRefGoogle ScholarPubMed
29. Hemnes, AR, Kawut, SM. The right ventricle in pulmonary hypertension: from dogma to data. Am J Respir Crit Care Med 2010; 182: 586–588.Google Scholar
30. Ryan, JJ, Archer, SL. The right ventricle in pulmonary arterial hypertension: disorders of metabolism, angiogenesis and adrenergic signaling in right ventricular failure. Circ Res 2014; 115: 176–188.Google Scholar
31. Jone, PN, Hinzman, J, Wagner, BD, Ivy, DD, Younoszai, A. Right ventricular to left ventricular diameter ratio at end-systole in evaluating outcomes in children with pulmonary hypertension. J Am Soc Echocardiogr 2014; 27: 172–178.Google Scholar