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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: [email protected]
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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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