Case presentation
A 53-years-old male patient from the Eastern Mediterranean Region with background of diabetes mellitus type II (DM II) and hypertension (HTN), both controlled with oral medications, presented to the primary health care center because of acute onset chest pain. Chest pain lasted for several minutes and was left-sided and non-radiating. There were no associated symptoms and no specific worsening or relieving factors. Family history is non-significant. On examination patient was afebrile, normal pulse and respiratory rates (HR 78, RR 16), blood pressure was high at 155/94 mmHg. Systemic examination was unremarkable. ECG (Figure 1 ) showed sinus rhythm with T-inversion in I, II, avl, V3-V6, which were considered old when compared to the ECG in 2017. The Patient was transferred rapidly to the emergency department (ED) for further evaluation and management through the emergency ambulance service.
In the ED, the patient was kept under observation, he was chest pain free with stable vital signs and physical examination. Chest radiographs and laboratory investigations were unremarkable including for two sets of high sensitivity cardiac troponin-T (Troponin T HS) with values of 7 ng/L and 8 ng/L respectively. Rest of the laboratory panel are as mentioned in table 1 . The patient was discharged with cardiology outpatient follow up advise.
A 2D transthoracic Echocardiography was done four days later during the outpatient cardiology clinic follow up visit, (Figure 2 ), which revealed; mildly reduced left ventricular (LV) systolic function with Ejection Fraction of 46 %, asymmetric LV hypertrophy affecting the apical segments which was aneurysmal. The apical cap contained a calcific material (calcified aneurysm vs calcified thrombus) and Grade 1 diastolic dysfunction were noted. Further evaluation with CT coronary angiogram showed non-significant left anterior descending artery lesion with concentric LV myocardial hypertrophy affecting the LV apex with subendocardial apical calcifications.
Cardiac MRI (CMR) images were performed later. Images obtained on a 1.5 tesla scanner (Philips Ingenia) revealed thickening of the akinetic LV apical segments (Figure 3 ) which demonstrated non-ischemic intense LV apical sub-endocardial late gadolinium enhancement (Figure 4 ). An apical LV thrombus was also noted. CMR concluded that the findings were consistent with EMF.
At this point, a diagnosis of EMF was made, and the patient was started on therapeutic dosing of anticoagulation in addition to Valsartan and Bisoprolol. Blood works were repeated, C-reactive protein level was 4.9 mg/L and ANCA and Anti-Schistosoma-Ab were negative. Peripheral smear revealed normochromic, normocytic RBC’s with few teardrop cells and mild rouleaux formation seen with a mild absolute eosinophilia and occasional reactive lymphocytes. The patient continued to have a non-exertional, non-radiating, on and off left sided chest pain, hence an adenosine stress CMR perfusion study was performed. This CMR performed almost 6 weeks after the initial CMR showed no stress induced perfusion defects, but there was regression of the size of LV apical thrombus with no other obvious interval significant changes.
Discussion: EMF is a rare and a newly emerging entity, it was first described in 1947 in Uganda. It is more commonly found in the developing world, such as Africa, Asia, and South America (1). it is characterized mainly by fibrosis of the apical endocardium mainly in the right, left or both ventricles. The clinical manifestations of it usually arise from the symptoms of heart failure secondary to restrictive LV filling (2). The symptoms depend on the extent of involvement of the ventricles. The initial phase of the disease is acute carditis, characterized by febrile illness and in some cases, it progresses to cardiogenic shock. But the vast majority present during the chronic phase with symptoms of heart failure, arrhythmia and/or thromboembolism (1, 4).
Generally, the main pathological event is unknown, however there are multiple theories about its development and further studies are needed to further unfold its etiologies and help us understand it in a better way. some of the theories that have been postulated are: 1- eosinophilia related process, which can lead to endocardial damage and fibrosis. 2- infection with prevalent organisms however no specific organism has been linked to its development. 3- autoimmune phenomenon, has also been considered as some studies have found anti-myosin antibodies, however finding these antibodies is not specific, and lastly 4- genetic components have been suggested as well, but none were completely linked to it (5). Diagnosis is usually challenging, as little is known about the true nature of the disease, however there are several supporting features that we can depend on, ECG is normal in most cases, however, in advanced cases it can show non-specific ST-T wave abnormalities, variable degrees of conduction block, or arrythmias (6). Complete blood count can show eosinophilia which can further support the diagnosis; but it is usually absent (7). Echocardiography is the modality of choice while evaluating for the disease, it can show apical obliteration, endocardial surface thrombi, or AV valve abnormalities (8). Other imaging modality that is currently gaining popularity is Cardiac MRI with gadolinium contrast enhancement which can demonstrate enhancement that further supports the diagnosis (9).
Treatment is often challenging, as there is little evidence on how to treat. Standard medical therapy is to be used, such diuretics for decongestion, ACEi⁄ARBs and beta blockers which can provide symptomatic relief, but the disease usually progresses in a short period of time. Anticoagulation is indicated for patients with proven endocardial thrombus (10). Surgery is one of the modalities that has improved survival in patients with advanced disease, the currently used approach includes endocardiectomy combined with valvular replacement if the valve is damaged, however it conveys a high mortality rate approaching 20%, in addition, recurrence after surgery has also been described. In severe advanced cases resistant to treatment, last resort is heart transplantation (11). Prognosis is usually poor due to the progressive nature of the disease and the increased risk for complications such as, arrythmias and sudden cardiac deaths (12).