Endomyocardial Fibrosis mimic a congenital heart disease; contrast cardiac magnetic resonance can differentiate the diagnosis

Description of Clinical Presentation: A 28-year- old female patient with no past medical history.
Symptoms:- One year ago
palpitation :- recurrent attacks of rapid and irregular palpitations
Shortness of breath with moderate exertion
Examination:-
Systolic murmur at lower sternal border , tachycardia and hepatomegaly.
Other systems examinations were unremarkable.

Diagnostic Techniques and Their Most Important Findings: 1- Labs:- with in normal , (CBC) with no eosinophilia.
2- ECG:- revealed Supraventricular tachycardia at 130 bpm.
3- CXR: showed cardiomegaly.
4- Transthoracic Echocardiography (TTE) showed:
- Hugely dilated right atrium.
- Apical displacement of the tricuspid valve with adherence of the septal leaflet to the myocardium (7.9 mm per m2) resulting in mal-coaptation and sever tricuspid regurgitation; suspect Ebstein anomaly.
- Right ventricular systolic function is impaired.
- Preserved left ventricular systolic function.
5- Patient was referred for Cardiac magnetic Resonance (CMR) for confirm diagnosis and accurate measures of left and right ventricles volume, Flow and function.
There are linear masses lining both ventricles. Right ventricular apex is partially obliterated. Tissue characterization of the masses shows that on T1W & T2W images, the masses have low signal. On perfusion images, the masses do not enhance. On delayed hyperenhancement images, the masses do not enhance, which indicates (with other features) thrombi.
- The right ventricle is small in size with normal systolic function. The right atrium is markedly dilated, with signs of right sided volume overload. - Apical displacement of septal leaflet of tricuspid valve (7.2mm per m2), but not fulfilling Ebstein anomaly criteria. However, there is Moderate to severe tricuspid regurgitation (Regurgitant fraction=45%).
- The left ventricle is normal in size. The left atrium is mildly dilated.
- Delayed Hyperenhancement findings (LGE):-It demonstrates circumferential endocardial hyperenhancement that affects less than 50% of the wall thickness. All affection is limited to the endocardial surface. Endocardial fibrosis is seen at whole LV segments, and extends into the right ventricle. Hence, the diagnosis consistent with biventricular Endomyocardial fibrosis
- Referred the patient for consultation of cardiovascular surgery.
- Regular follow and repeated CMR.

Learning Points from this Case: 1- CMR is the modality of choice in diagnosis of EMF due to:-
A- Defines endo-myocardium, extent of fibrosis and thrombosis.
B-Evaluation of the disease and follow up.
2- CMR unmask EMF from other common diseases eg, Apical hypertrophic cardiomyopathy, Constrictive pericarditis, Rheumatic valve disease and Epstein's anomaly.
3- Golden diagnostic test for EMF is endo-myocardial biopsy. However, CMR is alternative non invasive in diagnosis and follow up.

a) 4 Chamber SSFP view b, C) T1w and T2 w 4 chamber images d,e) Early and late gadolinium 4 chamber view.
SSFP 2chamber view
SSFP 4 chamber view