Echocardiography in sickle cell anaemia patients under 20 years of age: a descriptive study in the Brazilian Western Amazon.

INTRODUCTION: Cardiac abnormalities in sickle cell anaemia are frequent and early, despite being more evident in adulthood. The study on cardiac abnormalities is essential in the current context, as, owing to improved health, children are increasingly able to reach adulthood and suffering the consequences of chronic cardiac injury. OBJECTIVE: The aim of this study was to determine the prevalence of echocardiographic changes in patients under 20, suffering from sickle cell disease in Rio Branco, Brazilian Western Amazon. METHODS: The descriptive epidemiological study compare two sets of children and adolescents, one including sickle cell anaemia patients (n=45), and other one (n=109) without sickle cell anaemia or heart disease. The echocardiographic measurements were indexed according to body surface using z-scores, and the prevalence of echocardiographic changes in both groups, with their respective 95% confidence intervals, ascertained and compared. RESULTS: Compared with the non-sickle cell anaemia series, the sickle cell anaemia group showed z-scores 13.1-fold higher for the diastolic diameter of the left ventricle, 5.2 times higher for the thickness of the posterior wall, 4.9 higher for the left atrium, 2.5 times higher for the right ventricle and 2.0 times higher for the septum thickness. Also the rate of left ventricular mass, systolic pressure of the right ventricle and the relative wall thickness were significantly higher in sickle cell anaemia set. CONCLUSIONS: Cardiac abnormalities were observed in 93.5% of patients. Early detection of cardiac abnormalities and quantifying them using the indexation of echocardiographic measurements according to body surface will allow proper identification and attendance of these children.

Vivax malaria in an Amazonian child with dilated cardiomyopathy.

A child living in the Brazilian Amazon region who had had vivax malaria at the age of 11 months was admitted three months later with a history of progressive dyspnoea and fever, which culminated in respiratory distress and severe dilated cardiomyopathy at hospital admission in a malaria-free area. She received treatment for cardiac insufficiency and was tested for malaria with two thick blood smears, which were negative. There was general improvement of cardiorespiratory function in the next two weeks, but in the third week of hospital admission, there was re-appearance of fever, severe anaemia, severe plaquetopaenia, and respiratory distress. A third thick blood smear was positive for Plasmodium vivax mono-infection, which was confirmed by molecular methods. A serological panel with the most prevalent infectious agents known to cause myocarditis was performed, and specific anti-cytomegalovirus (CMV) IgM and elevated levels of anti-CMV IgG were also detected in the serum. After treatment for malaria, there was improvement of respiratory distress, although cardiac function did not recover. She was discharged home with drugs for cardiac insufficiency and is currently under follow-up with a paediatric cardiologist as an outpatient. This report presents a young child with several episodes of vivax malaria who suffers from cardiac insufficiency, probably related to CMV-induced myocarditis.