Ventricular diastolic dysfunction in sickle cell anemia is common but not associated with myocardial iron deposition.

Pediatric Blood & Cancer
Jane S HankinsVijaya Joshi

Abstract

Cardiac failure from myocardial iron deposition is a severe complication in patients with transfusion-related iron overload. Progressive heart damage from iron overload can cause left ventricular systolic and diastolic dysfunction in patients with hematologic disorders. Since nontransfused patients with sickle cell anemia (SCA) have a high incidence of diastolic dysfunction, we investigated the relationships among transfusional iron burden, myocardial iron deposition, and diastolic ventricular dysfunction by T2*-MRI and tissue Doppler echocardiography in iron-overloaded children with SCA. Children (> or =7 years) with SCA and iron overload (serum ferritin >1,000 ng/ml or > or =18 lifetime transfusions) were eligible. Serum ferritin and hepatic iron content (HIC) were measured and participants underwent nonsedated T2*-MRI of the heart, echocardiogram, electrocardiogram, and multi-uptake gated acquisition (MUGA) scan. Age-matched normative echocardiographic data were used for comparison. Among 30 children with SCA (median age, 13 years) and iron overload, mean (+/-SD) HIC and serum ferritin were 10.8 mg Fe/g (+/-5.9 mg Fe/g) and 3,089 ng/ml (+/-2,167 ng/ml), respectively. Mean T2*-MRI was 33 msec (+/-7 msec, range, 22-49). Echoca...Continue Reading

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