Systematic Reviews
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
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
Cláudio Lima Souza, Márcio Vasconcelos Oliveira, Fabrício Freire de Melo, Maria Luísa Cordeiro Santos, Ícaro Garcia Viana, Jamile Silva Lopes
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
Research background

Sickle cell anemia (SCA) is part of a group of hemoglobinopathies called sickle cell disease (SCD) in which individuals inherit hemoglobin variants derived from single-point mutations that result in morphological abnormalities in red blood cells.

Research motivation

Illness related to SCD is an important public health problem worldwide as it has a great impact on morbidity and mortality in the affected population which in Brazil is estimated at 30000 patients with an annual increase of 3500 new cases. Furthermore, about 20% of children do not reach the first 5 years of life, especially when they do not have adequate medical care.

Research objectives

This study aimed to systematically compile information about cardiopulmonary changes in patients with SCA.

Research methods

A systematic literature review was performed based on the PRISMA recommendation including scientific articles indexed in the Scientific Electronic Library Online databases, 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 only 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.

Research results

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.

Research conclusions

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.

Research perspectives

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.