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Echocardiography in sickle cell anaemia patients under 20 years of age: a descriptive study in the Brazilian Western Amazon

Published online by Cambridge University Press:  23 October 2013

Melissa C. V. Ribera ,
Ricardo B. Ribera ,
Rosalina J. Koifman  and
Sérgio Koifman
Melissa C. V. Ribera*
Affiliation:
Master’s Program in Public Health, Acre Federal University, Rio Branco, Acre, Brazil
Ricardo B. Ribera
Affiliation:
Children’s Hospital, Pediatric Cardiology Service, Rio Branco, Acre, Brazil
Rosalina J. Koifman
Affiliation:
Department of Epidemiology and Quantitative Methods in Health, National School of Public Health, Oswaldo Cruz Foundation, Ministry of Health of Brazil, Rio de Janeiro, RJ, Brazil
Sérgio Koifman
Affiliation:
Department of Epidemiology and Quantitative Methods in Health, National School of Public Health, Oswaldo Cruz Foundation, Ministry of Health of Brazil, Rio de Janeiro, RJ, Brazil
*
Correspondence to: M. C. V. Ribera, Department of Health Science and Sport, Acre Federal University, Rodovia BR 364, Km 4, Distrito Industrial, Rio Branco – Acre, Brazil. CEP 69.915-900. Tel: +55(68)84133005; Fax: +55(68)3901-1246; E-mail: vieira_mel@hotmail.com
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Abstract

Introduction

Cardiac abnormalities in sickle cell anaemia are frequent and early, despite being more evident in adulthood. The study on cardiac abnormalities is essential in the current context, as, owing to improved health, children are increasingly able to reach adulthood and suffering the consequences of chronic cardiac injury.

Objective

The aim of this study was to determine the prevalence of echocardiographic changes in patients under 20, suffering from sickle cell disease in Rio Branco, Brazilian Western Amazon.

Methods

The descriptive epidemiological study compare two sets of children and adolescents, one including sickle cell anaemia patients (n=45), and other one (n=109) without sickle cell anaemia or heart disease. The echocardiographic measurements were indexed according to body surface using z-scores, and the prevalence of echocardiographic changes in both groups, with their respective 95% confidence intervals, ascertained and compared.

Results

Compared with the non-sickle cell anaemia series, the sickle cell anaemia group showed z-scores 13.1-fold higher for the diastolic diameter of the left ventricle, 5.2 times higher for the thickness of the posterior wall, 4.9 higher for the left atrium, 2.5 times higher for the right ventricle and 2.0 times higher for the septum thickness. Also the rate of left ventricular mass, systolic pressure of the right ventricle and the relative wall thickness were significantly higher in sickle cell anaemia set.

Conclusions

Cardiac abnormalities were observed in 93.5% of patients. Early detection of cardiac abnormalities and quantifying them using the indexation of echocardiographic measurements according to body surface will allow proper identification and attendance of these children.

Keywords

Sickle cell anemiaechocardiographic alterationschildheart diseaseamazon
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 1 , January 2015 , pp. 63 - 69
Copyright
Copyright © Cambridge University Press 2013 

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References

1. Naoum, PC. Hemoglobinopatias e Talassemias. São Paulo: Sarvier; 1997: 137–143.Google Scholar
2. Silva, RBP, Ramalho, AS, Cassorla, RMS. A anemia falciforme como problema de saúde pública no Brasil. Rev Saúde Pública 1993; 27: 54–58.Google Scholar
3. Zago, MA, Costa, FF. Hereditary hemoglobin disorders in Brazil. Trans R Soc Trop Med Hyg 1995; 79: 385–388.CrossRefGoogle Scholar
4. Di Nuzzo, DVP, Fonseca, SF. Anemia falciforme e infecções. J Pediatria 2004; 80: 347–354.Google Scholar
5. Watanabe, AM. Prevalência de Anemia Falciforme no Estado do Paraná. 2007.122f. Dissertação (Mestrado) – Universidade Federal do Paraná, Curitiba, 2007.Google Scholar
6. BRASIL. Ministério da Saúde. Manual de Educação em Saúde. Secretaria de Atenção à Saúde, Departamento de Atenção Especializada (Health Ministry, Secretary of healthcare, Manual health education). Brasília: Ministério da Saúde 2009; v. 2: 13.Google Scholar
7. Ahmed, S, Siddiqui, AK, Sadiq, A, Shahid, RK, Patel, DV, Russo, LA. Echocardiographic abnormalities in sickle cell disease. Am J Hematol 2004; 76: 195–198.Google Scholar
8. Covitz, W, Esperland, M, Gallagher, D, Hellenbrand, W, Leff, S, Talner, N. The heart in sickle cell anemia: the cooperative study of sickle cell disease. Chest 1995; 108: 1214–1219.CrossRefGoogle ScholarPubMed
9. 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
10. Lester, LA, Sodt, PC, Hutcheon, N, Arcilla, RA. Cardiac abnormalities in children with sickle cell anemia. Chest 1990; 98: 1169–1174.Google Scholar
11. Grossman, W, Braunwald, E, Mann, T, McLaurin, LP, Green, LH. Contractile state of the left ventricle in man as evaluated from end-systolic pressure-volume relations. Circulation 1977; 56: 845–852.CrossRefGoogle Scholar
12. Batra, AS, Acherman, RJ, Wong, W, et al. Cardiac abnormalities in children with sickle cell anemia. Am J Hematol 2002; 70: 306–312.CrossRefGoogle ScholarPubMed
13. Hutz, MH. História natural da anemia falciforme em pacientes da região metropolitana do Rio de Janeiro (Tese Doutorado em Ciências). UFRGS, Porto Alegre, 1981: 275.Google Scholar
14. Martins, WA, Mesquita, ET., Cunha, DM, Pinheiro, LAF, Romêo, LJMF, Pareto, RC Jr. Estudo ecodopplercardiográfico em adolescentes e adultos jovens portadores de anemia falciforme. Arq Bras Cardiol 1999; 73: 463–468.Google Scholar
15. Estrade, G, Poitrineau, O, Bernasconi, F, Garnier, D, Donatien, Y. Fonction ventriculaire gauche et drépanocytose. Arch Mal Coeur 1989; 82: 1975–1981.Google Scholar
16. Herdy, GVH, Aguas, AFF, Chedid, TC. Alterações cardíacas na anemia falciforme. Arq Bras Cardiol 1983; 40: 311–315.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. Hassell, KL. Population estimates of sickle cell disease in the U.S. Am J Prev Med 2010; 38: S512–S521.Google Scholar
19. Lang, RM, Bierig, M, Devereux, RB, et al. 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 2005; 18: 1440–1463.Google Scholar
20. Daubeney, PEF, Blacksone, EH, Weintraub, RG, Slavik, Z, Scanlon, J, Webber, SA Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children. Cardiology in the Young 1999; 9: 402–410.Google Scholar
21. Tei, C, Nishimura, RA, Seward, JB, Tajik, AJ. Non-invasive Doppler derived myocardial performance índex: correlation with simultaneous measurements of cardiac catheterization measurements. J Am Soc Echocardiogr 1997; 10: 169–178.Google Scholar
22. Devereux, RB, Alonso, DR, Lutas, EM, et al. Echocardiographic assessment of left ventricular hypertrophy: comparison to necropsy findings. Am J Cardiol 1986; 57: 450–458.Google Scholar
23. Gladwin, MT, Sachdev, V, Jison, ML, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004; 350: 886–895.CrossRefGoogle ScholarPubMed
24. Ganau, A, Devereux, RB, Roman, MJ, et al. Patterns of left ventricular hypertrophy and geometric remodeling in essential hypertension. J Am Coll Cardiol 1992; 19: 1550–1558.Google Scholar
25. Paul, LW, Juhl, JH. Princípio de Interpretação Radiológica, 6th edn. Rio de Janeiro, Guanabara, 1980: 883.Google Scholar
26. Minniti, CP, Sable, C, Campbell, A, et al. Elevated tricuspid regurgitant jet velocity in children and adolescents with sickle cell disease: association with hemolysis and hemoglobin oxygen desaturation. Haematol 2009; 94: 340–347.CrossRefGoogle ScholarPubMed
27. Kato, GJ, Onyekwere, OC, Gladwin, MT. Pulmonary hypertension in sickle cell disease: relevance to children. Pediatric Hematol Oncol 2007; 24: 159–170.CrossRefGoogle ScholarPubMed