Left Ventricular Endomyocardial Fibrosis with Underlying Connective Tissue Disease Presenting as Left Upper Limb Monoparesis (Embolic Stroke)

Endomyocardial fibrosis is a restrictive cardiomyopathy of uncertain aetiology characterizsed by fibrin deposits over the endocardial surfaces of apices or inflow tract of either or both ventricles. We report here a case of thirty six years old female who presented to the emergency medical services with sudden onset left upper limb weakness. On enquiry she gave history of bilateral, symmetrical multiple joint pain (small and large) and hair loss since 8 years, loss of weight since 6 months, breathlessness on exertion and low grade fever since 1 month, non-exertional chest pain not associated with diaphoresis or palpitation since 15 days. There was no significant history of acute coronary syndrome in past.

On investigations, Chest X ray revealed cardiomegaly, 2 D Echo and Colour Doppler revealed layered homogenous mass attached to posterolateral left ventricle and extending to LV apex with anechoic region within, involving papillary muscle. There was associated restricted motion of left ventricular wall. Doppler showed mild MR. There was mild pericardial effusion.

Cardiac MRI revealed Lamellar thrombus along mid anteroseptal and posterolateral wall with no contrast enhancement and was isointense to myocardium on T1 and T2 w images hypointense to myocardium on BFFE images, subendocardial scarring of basal mid-anterior, antero-sepal and lateral wall of LV associated with hypokinesia on cine images.

In view of significant history of multiple (small and large) joint pain and hair loss her Anti-nuclear Antibody (ANA) test was done which was positive.

Endomyocardial biopsy revealed fibrin thrombus with entrapped neutrophils compatible with thrombotic stage of endomyocardial fibrosis.

Thus, we present a case of Endomyocardial Fibrosis (EMF) with connective tissue disorder.