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Number 10-07: Sludge Infarcts due to Homozygous Sickle Cell Anemia
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Number 10-07:  Sludge Infarcts due to Homozygous Sickle Cell Anemia

Case from:  Bansmann PM1, Stamm I1, Schneider M2, Beyer D1

Institution:  1KardioMR Cologne/Bonn, Koeln, Germany; 2German Pediatric Heart Center St. Augustin, Germany

Clinical history: 16 y/o male with known homozygous sickle cell anemia who presented with recurrent episodes of chest pain.  Electrocardiogram was normal without evidence of prior infarction.   He does have a history of aortic and mitral valve insufficiency with operative reconstruction of mitral valve in 2005 due to severe prolapse.  Echocardiogram at the time of mitral valve reconstruction demonstrated normal left ventricular function and no wall motion abnormalities.  There was no prior history of atrial septal defect, cocaine use, or myocarditis. The patient was referred for evaluation of possible ischemic myocardial damage.

Angiography:  Coronary angiography was unremarkable without any visible disease or luminal irregularities (Figures 1a and 1b). Left ventriculography revealed focal inferior and posterolateral wall hypokinesis (Movie 1).

 

 

Figure 1a

 

 

Figure 1b

 

 

Movie 1

 Cine CMR:  Regional akinesis of the inferior wall and hypokinesis of inferolateral myocardium were confirmed (Movie 2). Despite this finding, left ventricular ejection fraction was normal (LVEF=69%).

 

 

Movie 2

 

Delayed Enhancement:  Focal, partially subendocardial and transmural delayed enhancement was present within the basal and midventricular inferior wall segments. Additionally, apicolateral and anteroseptal subendocardial enhancement were also present (noted by arrows in Figure 2).

 

Figure 2

 

Conclusion:  Given this young individual's history of sickle cell disease, chest pain, and an unremarkable coronary angiography, the conclusion was drawn that the etioloogy of his wall motion abnormalities and focal subendocardial scar were due to red blood cell sickling and aggregation (sludge based myocardial infarction).

Perspective:  Myocardial fibrosis, be it through focal or diffuse microvascular obstruction, is a process that contributes to sickle cell cardiomyopathy.  The focal uptake of gadolinium associated with wall motion abnormalities and myocardial thinning suggests sludge infarcts which have also been demonstrated postmortem.  Diffuse fibrosis is difficult to detect due to the resolution of late gadolinium enhancement sequences and absense of wall thinning but there is suggestion that T1 mapping may be useful in this situation. 

References: 

1. James TN. Homage to James B. Herrick: A contemporary look at myocardial infarction and at sickle cell disease. Circulation. 2000 Apr;101:1874-1887.

2. Raman SV, Simonetti OP, Cataland SR, Kraut EH. Myocardial ischemia and right ventricular dysfunction in adult patients with sickle cell disease. Haematologica. 2006 Oct;91(10):1329-35.

3. Westwood MA, Shah F, Anderson LJ, Strange JW, Tanner MA, Maceira AM, Howard J, Porter JB, Walker JM, Wonke B, Pennell DJ. Myocardial tissue characterization and the role of chronic anemia in sickle cell cardiomyopathy. J Magn Reson Imaging. 2007 Sep;26(3):564-8.

 

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COTW handling editor: Kevin Steel

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