Cardiology and Cardiovascular Research

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Dilated Cardiopathy Associated with Sickle Cell Disease in a 68 Years Old Female: An Emerging Complication in Sub-sahara Africa

Received: 23 August 2019    Accepted: 09 September 2019    Published: 21 September 2019
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Abstract

Sickle Cell Anemia (SCA) is an autosomal recessive disease caused by a point mutation in the hemoglobin beta gene found on chromosome 11p15.5 [1]. Specifically, it occurs when a single base from A to T in the codon for glutamic acid at position 6 is changed to valine of the beta globin and thus disrupts the tertiary structure and stability of the hemoglobin molecule [2]. Sickle hemoglobin is responsible for wide spectrum of disorders which vary with respect to severity of anemia, frequency of crises and duration of survival [3]. We present the case of a dilated cardiopathy in an elderly female sickle cell patient. A 68 years old female sickle cell patient with no known major cardiovascular risk factor presented with progressive onset of dyspnea. Clinical examination showed signs of left ventricular failure; an electrocardiogram showed a sinus regular rhythm, left ventricular hypertrophy with systolic overload, Q waves in the anteroseptal leads. Cardiac ultrasound showed a dilated cardiopathy with a preserved left ventricular systolic function at 57%, normal regional wall motion and normal pulmonary pressure. We concluded of heart failure due to probable chronic anemia or anischemic cardiopathy with a conserved systolic function in an elderly sickle cell patient but we were not able to confirm the main etiology without CT Coronary Angiogram or coronarography. Sickle cell anemia is a common genetic condition in sub-Saharan Africa associated with early death. This case is special because we have an elderly female presenting with heart failure on a dilated cardiopathy. This enhances the necessity of strict cardiovascular follow up of Sickle cell patient.

DOI 10.11648/j.ccr.20190303.15
Published in Cardiology and Cardiovascular Research (Volume 3, Issue 3, September 2019)
Page(s) 65-70
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Sickle Cell Disease, Dilated Cardiopathy, Older Female, Sub-Sahara Africa

References
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[2] Reis F, Narmin A, Vladimir N. Understanding the role of intrinsic disorder in subunits of hemoglobin and the disease prosess of sickle cell anemia. Intrinsically Protein Disordered 2016.
[3] Hemalat L, Pradeep R, Rajesheka M. Spectrum of sickle cell disease im patients diagnosed at a tertiary care center in Karnataka with special emphasis on their Clincoheamatological profile. Journal of Clinical and Diagnostic Research 2016 10 (2) EC 09-EC11.
[4] Saunthararajah Y, Vichinsky EP. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Saunders Elsevier; 2012. Sickle Cell Disease: Clinical features and management. In: Hoffman R, Benz EJ Jr, Silberstein LE, Heslop HE, Weitz JI, editors. Ch. 40.
[5] Aziza S, Noah P. Sickle cell Disease. StatPearls Publishing 2019.
[6] Gossman W, Ehsan M, Maruvda S. Anemia, Sickle cell. StatPearls Publishing 2019.
[7] Gladwin M, Sachdev V. Cardiovascular Abnormalities in Sickle cell Disease. Journal of American College of Cardiology 2014 59 (13).
[8] Brousseau DC, Panepinto JA, Nimmer M, Hoffmann RG. The number of people with sickle-cell disease in the United States: national and state estimates. American Journal of Hematology. 2010; 85: 77–8.
[9] Julie M, Sharon E, Deogratias S, et al, Mortality in Sickle Cell Anemia in Africa: A prospective Cohort study in Tanzania. PLoS One 2011, 6 (2): e 14699.
[10] Prati D. Transmission of hepatitis C virus by blood transfusions and other medical procedures: a global review. Journal of Hepatology. 2006: 45 (4): 607-16.
[11] Kato GJ, Gladwin MT, Steinberg MH. Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Review. 2007; 21: 37–47.
[12] Platt OS, Brambilla DJ, Rosse WF et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994; 330 (23): 1639-44.
[13] Sophie Lanzkron, Patrick Carroll, Carlton Haywood. Mortality rayes and age at death from sickle cell disease: U.S., 1979-2005. Public Health Rep. 2013; 128 (2): 110-116.
[14] Graham R Serjeant. Mortality from sickle cell disease in Africa. BMJ. 2005; 330 (7489): 432-433.
[15] Ahmed S. Manifestations hépatiques et extra-hépatique liées au VHC. Hépato-Gastro., 2012, 19, (8): 649-60.
[16] Nicolas Goossens, Francesco Negro et al. Cardiovascular manifestations of hepatitis C virus. Clin Liver Dis. 2017. 1089-3261.
[17] Recommendations for prevention and control of hepatitis C virus (HCV) infection and HCV-related chronic disease. Centers for Disease Control and Prevention. MMWR Recomm Rep. 1998 Oct 16; 47 (RR-19): 1-39. PubMed | Google Scholar.
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Author Information
  • Department of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon; Department of Internal Medicine, Yaoundé University Teaching Hospital, Yaoundé, Cameroon

  • Department of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon

  • Department of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon

  • Department of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon

  • Department of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon

  • Department of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon

  • Department of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Yaoundé, Cameroon; Department of Internal Medicine, General Hospital of Yaoundé, Yaoundé, Cameroon

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    Sylvie Ndongo Amougou, Mary Anne Ngam, Murielle Helles Lema, Mazou Ngou Temgoua, Aicha Yap Mefire, et al. (2019). Dilated Cardiopathy Associated with Sickle Cell Disease in a 68 Years Old Female: An Emerging Complication in Sub-sahara Africa. Cardiology and Cardiovascular Research, 3(3), 65-70. https://doi.org/10.11648/j.ccr.20190303.15

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    ACS Style

    Sylvie Ndongo Amougou; Mary Anne Ngam; Murielle Helles Lema; Mazou Ngou Temgoua; Aicha Yap Mefire, et al. Dilated Cardiopathy Associated with Sickle Cell Disease in a 68 Years Old Female: An Emerging Complication in Sub-sahara Africa. Cardiol. Cardiovasc. Res. 2019, 3(3), 65-70. doi: 10.11648/j.ccr.20190303.15

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    AMA Style

    Sylvie Ndongo Amougou, Mary Anne Ngam, Murielle Helles Lema, Mazou Ngou Temgoua, Aicha Yap Mefire, et al. Dilated Cardiopathy Associated with Sickle Cell Disease in a 68 Years Old Female: An Emerging Complication in Sub-sahara Africa. Cardiol Cardiovasc Res. 2019;3(3):65-70. doi: 10.11648/j.ccr.20190303.15

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  • @article{10.11648/j.ccr.20190303.15,
      author = {Sylvie Ndongo Amougou and Mary Anne Ngam and Murielle Helles Lema and Mazou Ngou Temgoua and Aicha Yap Mefire and Anderson Ngouo Tchiffo and Samuel Kingue},
      title = {Dilated Cardiopathy Associated with Sickle Cell Disease in a 68 Years Old Female: An Emerging Complication in Sub-sahara Africa},
      journal = {Cardiology and Cardiovascular Research},
      volume = {3},
      number = {3},
      pages = {65-70},
      doi = {10.11648/j.ccr.20190303.15},
      url = {https://doi.org/10.11648/j.ccr.20190303.15},
      eprint = {https://download.sciencepg.com/pdf/10.11648.j.ccr.20190303.15},
      abstract = {Sickle Cell Anemia (SCA) is an autosomal recessive disease caused by a point mutation in the hemoglobin beta gene found on chromosome 11p15.5 [1]. Specifically, it occurs when a single base from A to T in the codon for glutamic acid at position 6 is changed to valine of the beta globin and thus disrupts the tertiary structure and stability of the hemoglobin molecule [2]. Sickle hemoglobin is responsible for wide spectrum of disorders which vary with respect to severity of anemia, frequency of crises and duration of survival [3]. We present the case of a dilated cardiopathy in an elderly female sickle cell patient. A 68 years old female sickle cell patient with no known major cardiovascular risk factor presented with progressive onset of dyspnea. Clinical examination showed signs of left ventricular failure; an electrocardiogram showed a sinus regular rhythm, left ventricular hypertrophy with systolic overload, Q waves in the anteroseptal leads. Cardiac ultrasound showed a dilated cardiopathy with a preserved left ventricular systolic function at 57%, normal regional wall motion and normal pulmonary pressure. We concluded of heart failure due to probable chronic anemia or anischemic cardiopathy with a conserved systolic function in an elderly sickle cell patient but we were not able to confirm the main etiology without CT Coronary Angiogram or coronarography. Sickle cell anemia is a common genetic condition in sub-Saharan Africa associated with early death. This case is special because we have an elderly female presenting with heart failure on a dilated cardiopathy. This enhances the necessity of strict cardiovascular follow up of Sickle cell patient.},
     year = {2019}
    }
    

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    AU  - Sylvie Ndongo Amougou
    AU  - Mary Anne Ngam
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    JO  - Cardiology and Cardiovascular Research
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