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Recurrent transfusion therapy in sickle cell disease - are there relevant echocardiographic differences in the evaluation of these patients?
Session:
Sessão de Posters 19 - Genética em Cardiologia 1
Speaker:
Ana Raquel Carvalho Santos
Congress:
CPC 2024
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
20. Congenital Heart Disease and Pediatric Cardiology
Subtheme:
20.6 Congenital Heart Disease – Clinical
Session Type:
Cartazes
FP Number:
---
Authors:
Ana Raquel Carvalho Santos; Isabel Cardoso; Inês Vieira; Chritopher Saunders; Madalena Silva; Tania Mano; Vera Ferreira; Pedro Rio; Ana Teresa Timoteo; Ana Galrinho; Patricia Ribeiro; Rui Cruz Ferreira
Abstract
<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Sickle cell disease (SCD) is the most common inherited blood disorder in the world. It is characterized by chronic hemolytic anemia. Life expectancy of patients (pts) with SCD is still reduced by more than 2 decades compared to the general population, and cardiovascular complications are a notable feature in the premature deaths. Identifying myocardial alteration at an early stage and improving management of this complication is an important step to improve quality of life and survival rate of the pts.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Aim: Evaluate differences in echocardiographic parameters of adult pts with SCD submitted to recurrent transfusion therapy (RTT)</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Methods: A retrospective, single centre analysis was made including pts with sickle cell disease referred to evaluation in hemoglobinopathies clinic. Echocardiogram was performed by Cardiology as part of usual follow up. Data were collected on population characteristics and echocardiographic measurements with definition of 2 groups differenced by the presence or absence of RTT. Descriptive statistics are presented as absolute frequency (number) and relative frequency (percentage) for categorical variables and as median and interquartile range (IQR) for continuous variables. When testing hypothesis, Mann-Whitney and Chi-Square tests were performed. A p value of 0.05 was considered statistically significant.</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Results:</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Data were collected of 128 pts followed in Hemoglobinopathies clinic with a median age was 31 years old (23-46). The majority were female, representing 53.9% of pts. Regarding comorbidities, 23.4% had a previous stroke, 11.7% hypertension, 3.1% diabetes, 17.2% kidney disease and 1.6% atrial fibrillation. RTT was necessary in 14.1%. When comparing echocardiographic differences between groups, indexed right atrium volume (20 mL/m2 Vs 35mL/m2, p=0.03), peak A velocity (80cm/s Vs 62 cm/s, p=0.00) and E/A ratio (1.2 Vs 1.9, p= 0.02) showed statistically significant differences between groups. Lateral e’, medial e’ and E/e’ ratio also showed differences between groups, without achieving statistically significance. There were no significant differences between groups regarding age, hypertension, and kidney disease. </span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Conclusion:</span></span></p> <p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Diastolic dysfunction in SCD is an independent risk factor for premature death. Our data shows statistically significant differences in diastolic dysfunction parameters in pts submitted to RTT, without differences between groups regarding age, hypertension, and kidney disease. </span></span></p>
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