Right and left ventricular function and myocardial scarring in adult patients with sickle cell disease: a comprehensive magnetic resonance assessment of hepatic and myocardial iron overload.
BACKGROUND
Patients with Sickle cell disease (SCD) who receive regular transfusions are at risk for developing cardiac toxicity from iron overload. The aim of this study was to assess right and left cardiac volumes and function, late gadolinium enhancement (LGE) and iron deposits in patients with SCD using CMR, correlating these values with transfusion burden, ferritin and hemoglobin levels.


METHODS
Thirty patients with SCD older than 20 years of age were studied in a 1.5 T scanner and compared to age- and sex-matched normal controls. Patients underwent analysis of biventricular volumes and function, LGE and T2* assessment of the liver and heart.


RESULTS
When compared to controls, patients with SCD presented higher left ventricular (LV) volumes with decreased ejection fraction (EF) with an increase in stroke volume (SV) and LV hypertrophy. The right ventricle (RV) also presented with a decreased EF and hypertrophy, with an increased end-systolic volume. Although twenty-six patients had increased liver iron concentrations (median liver iron concentration value was 11.83 ± 9.66 mg/g), only one patient demonstrated an abnormal heart T2* < 20 msec. Only four patients (13%) LGE, with only one patient with an ischemic pattern.


CONCLUSIONS
Abnormal heart iron levels and myocardial scars are not a common finding in SCD despite increased liver iron overload. The significantly different ventricular function seen in SCD compared to normal suggests the changes in RV and LV function may not be due to the anemia alone. Future studies are necessary to confirm this association.
Clínica de Diagnóstico Por Imagem (CDPI), Av, das Américas 4666 sala 325, Barra da Tijuca, Rio de Janeiro 22649-900RJ, Brazil. junqueira.fp@gmail.com.
Flávia P
FP
Junqueira
Juliano L
JL
Fernandes
Guilherme M
GM
Cunha
Tadeu
T
T A Kubo
Claudio
C
M A O Lima
Daniel
D
B P Lima
Marly
M
Uellendhal
Sidney R
SR
Sales
Carolina
C
A S Cunha
Viviani
V
L R de Pessoa
Clarisse
C
L C Lobo
Edson
E
Marchiori
D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications*; D016022:Case-Control Studies; D005260:Female; D005293:Ferritins / Q000097:blood; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000097:blood / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D017380:Hypertrophy, Right Ventricular / Q000097:blood / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D008099:Liver / Q000378:metabolism*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism / Q000473:pathology*; D011237:Predictive Value of Tests; D011446:Prospective Studies; D013318:Stroke Volume; D018487:Ventricular Dysfunction, Left / Q000097:blood / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D018497:Ventricular Dysfunction, Right / Q000097:blood / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right; D055815:Young Adult
D006454:Hemoglobins; D005293:Ferritins