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