Systematic Reviews
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World J Hematol. Jul 29, 2022; 9(3): 20-29
Published online Jul 29, 2022. doi: 10.5315/wjh.v9.i3.20
Cardiopulmonary changes in patients with sickle cell anemia: A systematic review
Jamile Silva Lopes, Ícaro Garcia Viana, Maria Luísa Cordeiro Santos, Fabrício Freire de Melo, Márcio Vasconcelos Oliveira, Cláudio Lima Souza
Jamile Silva Lopes, Ícaro Garcia Viana, Maria Luísa Cordeiro Santos, Fabrício Freire de Melo, Márcio Vasconcelos Oliveira, Cláudio Lima Souza, Instituto Multidisciplinar em Saúde, Universidade Federal da Bahia, Vitória da Conquista 45029-094, Bahia, Brazil
Author contributions: All authors contributed equally to this paper with conception and design of the study, literature review and analysis, drafting and critical revision, editing and final approval of the final version.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Fabrício Freire de Melo, PhD, Adjunct Professor, Instituto Multidisplinar em Saúde, Universidade Federal da Bahia, Rua Hormindo Barros, 58, Quadra 17, Lote 58, Vitória da Conquista 45029-094, Bahia, Brazil.
Received: August 30, 2021
Peer-review started: August 30, 2021
First decision: December 2, 2021
Revised: December 13, 2021
Accepted: July 8, 2022
Article in press: July 8, 2022
Published online: July 29, 2022

Given the high prevalence of cardiovascular and pulmonary abnormalities associated with sickle cell anemia (SCA), the clinical impact caused in addition to compromising the quality of life of patients and the overcharge that it represents to the public health system, this study systematized and evaluated scientific publications on pulmonary complications and cardiovascular diseases in sickle cell patients from 1920 to 2020. This compilation aims to provide knowledge for health professionals and managers in order to draw attention to the importance of chronic diseases in SCA patients and in addition to providing elements that provide improvements in management of useful resources that contribute to improve the quality and increase the life expectancy of these patients.


To systematically compile information about cardiopulmonary changes in patients with SCA.


A systematic literature review was performed based on the PRISMA recom-mendation including scientific articles indexed in the Scientific Electronic Library Online databases of the United States National Library of Medicine and Biblioteca Virtual de Saúde. The search period was delimited between 1990 and 2020 and selected in Portuguese, English and Spanish. Three sets of descriptors were used for each database including research carried out with human beings. After reading the articles, those useful for this review were extracted using a collection instrument designed for this purpose.


The final selection included 27 studies. The year with the highest number of publications was 2016 with 5 studies (18.51%), followed by 2017 with 4 (14.81%). The type of study most carried out in the period was cohort 10 (37.03%) followed by cross-sectional and case-control with 8 studies in each (29.62%). Regarding the language of publication, the distribution was as follows: 25 (92.59%) in English, 1 (3.70%) in Spanish and 1 (3.70%) in Portuguese.


The findings of the present study suggest that cardiopulmonary alterations represent a serious clinical repercussion of SCA. Of the analyzed studies, the high occurrence of pulmonary hypertension, ventricular hypertrophy and diastolic dysfunction stands out as the main cardiopulmonary complications. In view of the increased survival in SCA, there is a need for surveillance and the development of strategies aimed at preserving the cardiopulmonary function and consequently improving the quality of life of these patients.

Keywords: Sickle cell anemia, Cardiopulmonary alterations, Clinical profile, Systematic review

Core Tip: Sickle cell anemia (SCA) is the most common and severe form of sickle cell disease (SCD) accounting for approximately 70% of SCD cases worldwide. Illness related to SCA is an important public health problem as it is a serious chronic disease with limited possibility of cure and that causes suffering to its patients. With adult age and aging, cardiopulmonary changes are mainly observed. Given their high prevalence and the clinical impact caused to patients with SCA, this study compiled information about cardiopulmonary changes in patients with SCA.