Background and Aims:
Left ventricular noncompaction (LVNC) is a rare cardiomyopathy. It is most commonly congenital, however acquired forms have been described. It results in multiple and excessive trabeculations with deep intertrabecular recesses which communicate with the left ventricle (LV) and a thickened ventricular myocardium with two distinct layers, compacted and noncompacted. It varies in presentation, from severe prenatal manifestations to asymptomatic cardiomyopathy or even heart failure and systemic embolisation, secondary to mural thrombi formation. It has been reported to be more prevalent in individuals with β-thalassaemia, though the causative relationship has not to date been established.

Methods:
Case report

Results:
A 36-year-old male with β-thalassaemia intermedia presented with confusion and visual loss. He had splenectomy in childhood resulting in chronic moderate thrombocythaemia but was not transfusion-dependent with no hospitalisations as an adult.  Fundoscopy demonstrated retinal emboli. There were no clinical signs of congestive heart failure.  Brain magnetic resonance imaging (MRI) showed multi-territorial shower emboli. An echocardiogram showed restrictive LV filling pattern and calcification of the LV outlet. Cardiac MRI demonstrated features consistent with LVNC but ruled out significant cardiac siderosis. Other causes for calcification were ruled out. He was anticoagulated with warfarin but had recurrent, symptomatic retinal ischaemia. He has had no further events after the addition of clopidogrel.

Conclusions:
The co-existence of splenectomy-related thrombocythaemia with the deep intertrabecular recesses of LVNC may explain the recurrent embolism in this case, who did not have severe cardiac siderosis. Further research is needed to understand the link between LVNC and thalassaemia.

A RARE CAUSE OF EMBOLIC STROKE IN A PATIENT WITH Β-THALASSAEMIA