Author + information
- Rory O'Hanlon, MD⁎ (, )
- Grant Heatlie, MD,
- Ferrah Jabeen Choudhury, MD,
- Susanna Price, MD,
- Sanjay Prasad, MD and
- Mary Sheppard, MD
- ↵⁎Consultant Cardiologist, St. Vincent's University Hospital, Suite 12, Blackrock Clinic, CMR Unit, Royal Brompton Hospital, London, SW3 6NP, United Kingdom
Histopathological correlations with antemortem imaging findings in nonischemic cardiomyopathies, even though important for a deeper insight, are rare. These types of correlation are even rarer for the more uncommon entities (1). We aimed to validate antemortem echocardiography and contrast-enhanced cardiovascular magnetic resonance images with post-mortem pathology for the detection of thrombus and fibrosis in a 17-year-old man who presented with heart failure due to endomyocardial fibrosis. Echocardiography demonstrated normal left ventricular dimensions with low normal left ventricular systolic function, mitral valve regurgitation due to posterior leaflet restriction, and an echogenic mass consistent with endocardial thickening originating from the inferolateral and anterolateral wall with a superimposed thrombus (Figs. 1A and 1B). Cardiac magnetic resonance (CMR) with gadolinium contrast was performed and demonstrated near circumferential late enhancement of the endocardium from mid-ventricle to apex, with sparing of the septum with a hypointense superficial overlying thrombus (Figs. 1C and 1D). These findings were characteristic of endomyocardial fibrosis. The patient unfortunately passed away and autopsy revealed a large yellow necrotic mass lining the left ventricle, with an adherent fresh thrombus on the surface (Fig. 1E). Early and late contrast CMR detection of thrombus and fibrosis were thus closely correlated with macroscopic post-mortem findings. Microscopy confirmed dense replacement fibrosis in the regions defined by late contrast enhancement on CMR (Fig. 1F). This case represents a rare opportunity to correlate antemortem multimodality imaging findings with post-mortem validation in a case of idiopathic endomyocardial fibrosis, a condition that, though rare in the Western world, remains a common cause of restrictive cardiomyopathy in sub-Saharan Africa, Asia, Brazil, and India (1).
Drs. Heatlie and Choudhury are from the University Hospital of North Staffordshire, Stoke on Trent, United Kingdom; Drs. Price, Prasad, and Sheppard are from the Royal Brompton Hospital, London, United Kingdom. Research support was received from the National Institutes of Health Cardiovascular Biomedical Research Unit, BHF, and CORDA.
- American College of Cardiology Foundation