sickle cell anemia – ABC CARDIOL

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Editorial

Lipids and Sickle Cell Disease

Sickle cell disease is a hereditary, autosomal recessive pathology caused by the replacement of adenine by thymin in position 6 of the beta globin gene, producing anomalous hemoglobin (hemoglobin S- HbS). It is characterized by a chronic inflammatory state with hemolytic anemia and vaso-occlusive phenomena and occurs when the HbS gene is in homozygosis (SS) or heterozygosis, in association with other variant hemoglobins (SD, SC, SE) or thalassemia (). Sickle cell disease is recognized by the World Health Organization (WHO) as a serious public health problem, and, despite advances in treatment, patients with sickle cell disease have a life expectancy of nearly 30 years lower than the general population.

About 4% of the Brazilian population has a sickle cell trait, and between 60,000 and 100,000 Brazilians have the disease, which translates into an economic burden of more than US$ 400,000,000/year for the Brazilian society. The number of patients affected by this pathology is increasing due to the evolution of treatment and improvement in early diagnosis, as a result of the institution of neonatal screening. Early identification of the disease is important in reducing its morbidity and mortality, especially with the prophylaxis of infections, which are a significant cause of death in children. This improvement in survival leads to the development of chronic complications resulting from vasculopathy and to more frequent cases of chronic ischemia.

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Original Article

Doppler echocardiographic study in adolescents and young adults with sickle cell anemia

Wolney de Andrade Martins et al. Evandro Tinoco Mesquita Delma Maria da Cunha Luiz Augusto de Freitas Pinheiro … Luiz José Martins Romêo Fº Raul Carlos Pareto Jr

OBJECTIVE: Anatomical and functional assessment of the heart through Doppler and echocardiography in patients with cell anemia (SCA). METHODS: Twenty-five patients with SCA and ages ranging from 14 to 45 years were prospectively studied in a comparison with 25 healthy volunteers. All of them underwent clinical and laboratory evaluation and Doppler echocardiography as well.The measurements were converted into body surface indices. RESULTS: There were increases in all chamber diameters and left ventricle (LV) mass of the SCA patients. It was characterised an eccentric hypertrophy of the left ventricle. The preload was increased (left ventricle end-diastolic volume) and the afterload was decreased (diastolic blood pressure, peripheral vascular resistance and end-systolic parietal stress ESPS). The cardiac index was increased due to the stroke volume. The ejection fraction and the percentage of the systolic shortening , as well as the systolic time intervals of the LV were equivalent. The isovolumetric contraction period of the LV was increased. The mitral E-septum distance and the end-systolic volume index (ESVi) were increased. The ESPS/ESVi ratio,a loading independent parameter, was decreased in SCA, suggesting systolic dysfunction. No significant differences in the diastolic function or in the pulmonary pressure occurred. CONCLUSION: Chamber dilations, eccentric hypertrophy and systolic dysfunction confirm the evidence of the literature in characterizing a sickle cell anemia cardiomyopathy.

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Lipids and Sickle Cell Disease

Doppler echocardiographic study in adolescents and young adults with sickle cell anemia

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