Hostname: page-component-78c5997874-g7gxr Total loading time: 0 Render date: 2024-11-10T05:44:15.916Z Has data issue: false hasContentIssue false
  • English
  • Français

Cardiovascular changes in children with sickle cell crisis

Published online by Cambridge University Press:  24 January 2020

Richard Onalo Open the ORCID record for Richard Onalo [Opens in a new window] ,
Peter Cooper ,
Antoinette Cilliers  and
Uche Nnebe-Agumadu
Richard Onalo*
Affiliation:
Paediatric Cardiology Unit, Department of Paediatrics, Faculty of Clinical Sciences, University of Abuja, Abuja, Nigeria
Peter Cooper
Affiliation:
Department of Paediatrics, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa
Antoinette Cilliers
Affiliation:
Paediatric Cardiology Unit, Department of Paediatrics, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg, South Africa
Uche Nnebe-Agumadu
Affiliation:
Paediatric Haematology Unit, Department of Paediatrics, University of Abuja Teaching Hospital, Abuja, Nigeria
*
Author for correspondence: Richard Onalo, Department of Paediatrics, Faculty of Clinical Sciences, College of Health Sciences, University of Abuja, PMB 117, Gwagwalada, Abuja, Nigeria. Tel: +234 803 701 7678; E-mail: richardonalo@yahoo.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Background:

Sickle cell anaemia is characterised by frequent, sometimes serious events referred to as “crisis”. Cardiopulmonary consequences such as pulmonary hypertension and myocardial ischaemia may accompany a serious crisis.

Objective:

To determine the cardiovascular changes that occur during a severe sickle cell crisis.

Methods:

A cross-sectional comparative study of sickle cell anaemia in children (5–17 years) admitted during a severe crisis (cases) and those in steady state (controls) was conducted over a 2-year period. Effects of the crisis on the cardiopulmonary system were assessed. The diagnosis of myocardial ischaemia was made using electrocardiography and serological cardiac biomarkers, while cardiac dysfunction and the presence of pulmonary hypertension were determined using echocardiography. The presence of systemic hypertension and tachycardia was also evaluated.

Results:

A total of 176 patients were recruited, 92 in steady state (male:female ratio, 1.2:1) and 84 in severe crisis (male:female ratio, 1.3:1). The mean age was 10.4 ± 3.2 years for steady state and 10.5 ± 3.4 years for those in crisis. The mean heart rate in crisis was higher than in steady state (p < 0.0001). The blood pressures (systolic, p < 0.0001, diastolic, p < 0.0001, mean, p < 0.0001) as well as myocardial ischaemia scores (p < 0.0001) were higher in patients with crisis than in those in steady state. Similarly, conduction abnormalities, pulmonary hypertension, and ventricular dysfunction were more prevalent in the crisis than in the steady state.

Conclusion:

The present data suggest that sickle cell crisis results in a derangement of clinical, electrocardiographical, and echocardiographical parameters in children with sickle cell anaemia. Further research on these cardiovascular events may improve the overall care of these patients.

Keywords

Heart changespulmonary changessickle cell crisisNigerian children
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 2 , February 2020 , pp. 162 - 170
Copyright
© Cambridge University Press 2020

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Ashley-Koch, A, Yang, O, Olney, RS.Sickle hemoglobin allele and sickle cell disease: a HuGE review. Am J Epidemiol 2000; 151: 839–845.10.1093/oxfordjournals.aje.a010288CrossRefGoogle ScholarPubMed
Piel, FB, Patil, AP, Howel, RE, et al.Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nat Commun 2010; 1: 104.10.1038/ncomms1104CrossRefGoogle ScholarPubMed
CDC Global Health Nigeria: Sickle cell disease Atlanta: Centers for Disease Prevention and Control, 2012. Available at http://www.cdc.gov/globalhealth/countries/nigeria/what/scd.htm.Google Scholar
Powars, DR, Chan, LS, Hiti, A, Ramicone, E, Johnson, C.Outcome of sickle cell anemia: a 4-decade observational study of 1056 patients. Medicine (Baltimore) 2005; 84: 363–376.10.1097/01.md.0000189089.45003.52CrossRefGoogle ScholarPubMed
Elenga, N, Cuadro, E, Martin, E, Cohen-Addad, N, Basset, T.Associated factors of acute chest syndrome in children with sickle cell disease in French Guiana. Int J Pediatr 2014; 2014: Article ID 213681. doi: 10.1155/2014/213681.CrossRefGoogle ScholarPubMed
Alkali, MB, Ambe, JP.Acute chest syndrome in paediatric patients in Northeastern Nigeria. Direct Res J Hlth Pharm 2015; 3: 45–50.Google Scholar
Machado, RF, Kyle Mack, A, Martyr, S, et al.Severity of pulmonary hypertension during vaso-occlusive pain crisis and exercise in patients with sickle cell disease. Br J Haematol 2007; 136: 319–325.10.1111/j.1365-2141.2006.06417.xCrossRefGoogle ScholarPubMed
Dessap, AM, Leon, R, Habibi, A, et al.Pulmonary hypertension and CorPulmonale during severe acute chest syndrome in sickle cell disease. AJRCCM 2008; 177: 646–653.doi: 10.1164/rccm.200710-1606OC.Google Scholar
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: 886–895.10.1056/NEJMoa035477CrossRefGoogle ScholarPubMed
Gladwin, MT, Vichinsky, E.Pulmonary complications of sickle cell disease. N Engl J Med 2008; 359: 2254–2265.10.1056/NEJMra0804411CrossRefGoogle ScholarPubMed
Kuma, AB, Owusu-Amsah, AT, Ampomah, MA, et al.Prevalence of relative systemic hypertension in adults with sickle cell disease in Ghana. PLoS ONE 2018; 13: e0190347.10.1371/journal.pone.0190347CrossRefGoogle Scholar
Yusuf, HR, Atrah, HK, Grosse, SD, Parker, CS, Grant, AM. Emergency department visits made by patients with sickle cell disease: a descriptive study, 1999-2007. Am J Prev Med 2010; 38: S536–S541.10.1016/j.amepre.2010.01.001CrossRefGoogle ScholarPubMed
Ahmed, SG, Kagu, MB, Abjah, UA, Bukar, AA.Seasonal variations in frequencies of acute vaso-occlusive morbidities among sickle cell anaemia patients in Northern Nigeria. J Blood Disord Transfus 2012; 3: 120. doi: 10.4172/21559864.1000120.CrossRefGoogle Scholar
Vichinsky, EP, Neumayr, LD, Earles, AN, et al.Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000; 342: 1855–1865.10.1056/NEJM200006223422502CrossRefGoogle ScholarPubMed
Bode-Thomas, F, Hyacinth, HI, Ogunkunle, O, Omotoso, A.Myocardial ischaemia in sickle cell anaemia: evaluation using a new scoring system. Ann Trop Paediatr 2011; 31: 67–74.10.1179/1465328110Y.0000000006CrossRefGoogle ScholarPubMed
de Montalembert, M, Maunoury, C, Acar, P, Brouser, Sidi D, Lenoir, G.Myocardial ischaemia in children with sickle cell disease. Arch Dis Child 2004; 89: 359–362.10.1136/adc.2003.027326CrossRefGoogle ScholarPubMed
National High Blood Pressure Education Program Working Group on High blood pressure in children and adolescent. The fourth report on the diagnosis, evaluation and treatment of high blood pressure in children and adolescents. Pediatrics 2004; 114: 555–576.10.1542/peds.114.2.S2.555CrossRefGoogle Scholar
The Society for Cardiological Science and Technology. Clinical guidelines by consensus. Recording a standard 12-lead electrocardiogram. An approved methodology, 2010. Reviewed date Feb 2013.Google Scholar
Kolawole, AF, Omokhodion, SI.Normal limits for pediatric electrocardiogram in Ilorin, Nigeria. Nig J Cardiol 2014; 11: 112–123.10.4103/0189-7969.142103CrossRefGoogle Scholar
Rijnbeek, PR, Witsenburg, M, Schrama, E, Hess, J, Kors, JA.New normal limits for the paediatric electrocardiogram. Eur Heart J 2001; 22: 702–711.10.1053/euhj.2000.2399CrossRefGoogle ScholarPubMed
Park, MK, Guntheroth, WG.How to Read pediatric ECGs, 4th edn. Mosby Elsevier Inc, Philadelphia, 2006: 35–121.Google Scholar
Lai, WW, Geva, T, Shirali, GS, et al.Guidelines and standards for performance of a pediatric echocardiogram: a report from the task force of the pediatric council of the American Society of Echocardiography. J Am Soc Echocardiogr 2006; 19: 1413–1430.10.1016/j.echo.2006.09.001CrossRefGoogle ScholarPubMed
Abbas, AE, Fortuin, FD, Schiller, NB, Appleton, CP, Moreno, CA, Lester, SJ.A simple method for noninvasive estimation of pulmonary vascular resistance. J Am Coll Cardiol 2003; 41:1021–1027.10.1016/S0735-1097(02)02973-XCrossRefGoogle ScholarPubMed
Porter, TR, Shillcutt, SK, Adams, MS, et al.Guidelines for the use of echocardiography as a monitor for therapeutic intervention in adults: a report from the American Society of Echocardiography. J Am Soc Echocardiogr 2015; 28: 40–5610.1016/j.echo.2014.09.009CrossRefGoogle ScholarPubMed
Mortens, LL, Friedberg, MK.Echocardiographic assessment of cardiac dimensions, cardiac function and valve function. In: Allen, HD, Driscoll, DJ, Shaddy, RE, Feltes, TF (eds). Moss and Adams’ Heart Disease in Infants, Children, and Adolescents, 8th edn. Lippincott Williams & Wilkins, Philadelphia, 2013: 172–206.Google Scholar
Koestenberger, M, Nagel, B, Ravekes, W, et al.Left ventricular long-axis function: Reference values of the mitral annular plane systolic excursion in 558 healthy children and calculation of z-score values. Am heart J 2012; 164: 125–131.10.1016/j.ahj.2012.05.004CrossRefGoogle ScholarPubMed
Koestenberger, M, Ravekes, W, Everett, AD, et al.Right ventricular functions in infants, children and adolescents: Reference values of the tricuspid annular plane systolic excursion in 640 healthy patients and calculation of z-score values. J Am Soc Echocardiol 2009; 22: 715–719.10.1016/j.echo.2009.03.026CrossRefGoogle ScholarPubMed
Johnson, CS, Giorgio, AJ.Arterial blood pressures in adult with sickle cell disease. Arch Intern Med 1981; 144: 891–893.10.1001/archinte.1981.00340070071015CrossRefGoogle Scholar
Hatch, FE, Crowe, LR, Miles, DE.Altered vascular reactivity in sickle hemoglobinopathy: a possible protective factor from hypertension. Am J Hypertens 1989; 2: 2–8.10.1093/ajh/2.1.2CrossRefGoogle ScholarPubMed
Singer, M, Boghossians, S, Bevan, DH, Bennett, ED.Hemodynamic changes during sickle cell crisis. Am J Card 1989; 64: 1211–1213.10.1016/0002-9149(89)90884-9CrossRefGoogle ScholarPubMed
Emst, AA, Weiss, SJ, Johnson, WD, Takakuwa, KM.Blood pressure in acute vaso-occlusive crises of sickle cell disease. South Med J 2000; 93: 590–592.Google Scholar
Richards, CA, Tzeng, Y.Arterial pressure and cerebral blood flow variability: friend or foe? A review. Front Physiol 2014; 5: 120. doi: 10.3389/fphys.2014.00120.Google Scholar
Norris, S, John, CS, Haywood, LJ.Sickle cell anaemia: does myocardial ischaemia occur during crisis? J Natl Med Assoc 1991; 83: 209–213.Google ScholarPubMed
Maisel, A, Friedman, H, Flint, L, Koshy, M, Prabhu, R.Continuous electrocardiographic monitoring in patients with sickle cell anemia during crisis. Clin Cardiol 1983; 6: 339–344.10.1002/clc.4960060707CrossRefGoogle ScholarPubMed
Gordeuk, VR, Sachdev, V, Taylor, J, Gladwin, MT, Kato, G, Castro, O.Relative systemic hypertension in patients with sickle cell disease is associated with risk of pulmonary hypertension and renal insufficiency. Am J Hematol 2008; 83: 15–18.10.1002/ajh.21016CrossRefGoogle ScholarPubMed
MekontsoDessap, A, Leon, R, Habibi, A, et al.Pulmonary hypertension and corpulmonale during severe acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med 2008; 177: 646–653.10.1164/rccm.200710-1606OCCrossRefGoogle Scholar
Castro, O, Hoque, M, Brown, BD.Pulmonary hypertension in sickle cell disease: cardiac catherization results and survival. Blood 2002; 101: 1257–1261.10.1182/blood-2002-03-0948CrossRefGoogle Scholar
Supplementary material: File

Onalo et al. supplementary material

Appendix

Download Onalo et al. supplementary material(File)
File 20 KB