Cardiology in the Young

Original Articles

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

Melissa C. V. Riberaa1 c1, Ricardo B. Riberaa2, Rosalina J. Koifmana3 and Sérgio Koifmana3

a1 Master’s Program in Public Health, Acre Federal University, Rio Branco, Acre, Brazil

a2 Children’s Hospital, Pediatric Cardiology Service, Rio Branco, Acre, Brazil

a3 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


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.

(Received December 05 2012)

(Accepted September 08 2013)

(Online publication October 23 2013)


  • Sickle cell anemia;
  • echocardiographic alterations;
  • child;
  • heart disease;
  • amazon


c1 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: