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\begin{document}

\title{Endomyocardial Fibrosis in non-tropical patient with no obvious
eosinophilia presented with chest pain mimicking ACS and left
ventricular thrombus}



\author[1]{ahmad matarneh}%
\author[2]{Yousef Hailan}%
\author[2]{Sabir  Abdulkarim}%
\author[2]{Maryam  Alkuwari}%
\author[2]{Wafer  dabdoob}%
\affil[1]{Hamad medical corporation}%
\affil[2]{Hamad Medical Corporation}%


\vspace{-1em}



  
  \date{May 20, 2024}


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\selectlanguage{english}
\begin{abstract}
Endomyocardial fibrosis is a disease that causes restrictive
cardiomyopathy, it varies in presentation ranging from asymptomatic to
full-blown heart failure. its pathogenesis is poorly understood and it
poses a diagnostic and treatment challenge due to its infrequent
occurrence.%
\end{abstract}%



\sloppy


\textbf{Endomyocardial Fibrosis in non-tropical patient with no obvious
eosinophilia presented with chest pain mimicking ACS and left
ventricular thrombus}

Ahmad S Matarneh1 , Yousef M Ali Hailan1, Sabir Abdul Karim2, Maryam A
Al Kuwari3 Wafer A Dabdoob2

1.Internal Medicine, Department of Medical Education, Hamad Medical
Corporation (HMC), Qatar.

\selectlanguage{ngerman}¹Consultant Cardiologist, Department of Cardiology, Heart Hospital, HMC,
Qatar.

\selectlanguage{english}?Senior Consultant Radiologist, Chairman of the Department of Radiology,
Heart Hospital, HMC, Qatar.

\textbf{Corresponding Author:}

Dr. Ahmad Samir Salam Matarneh.

Department Internal Medicine.

Hamad Medical Corporation.

Al Rayyan street.

Doha, Qatar.

Tel: +97455957396

Fax: --44397857

E-mail: AMatarneh@Hamad.qa

\textbf{Key clinical message:}

Endomyocardial fibrosis is a rare cause of restrictive cardiomyopathy,
its pathology is poorly understood, and further theories are proposed as
a cause. Diagnosis and its treatment are challenging, and further
studies are needed to expand on it.

\textbf{Abstract}

Endomyocardial fibrosis (EMF) is a disease that known to cause
restrictive cardiomyopathy. It shows high prevalence in tropical
country. Several triggering factors has been proposed, however, the
pathogenesis is still mystery. The disease is progressive in nature and
the outcome is generally unfavorable. Most common symptoms is heart
failure, however, atypical presentation may expected.

Our case presented with symptoms suggestive of ischemia and missed
diagnosed initially as ischemic cardiomyopathy. The aim of this report
is to increase the attention and awareness for this disease.

We present a case of a 53-year-old man referred to emergency department
for sudden chest pain, left sided and non-radiating lasted for several
minutes, awoke him from sleep with no associated symptoms. He is known
to have Diabetes type-2 and hypertension on oral therapy. Cardiac
markers were within normal limit. The patient discharged home with
appointment to cardiology out-patient clinic. Echocardiography done and
revealed mildly reduced left ventricular (LV) systolic function Ejection
Fraction of 46 \%, asymmetric LV hypertrophy affecting the apical
segments with aneurysm and calcified apical thrombus. CT coronary
angiography done with non-significant Left Anterior Descending artery
lesions and left ventricular hypertrophy affecting the apex with
calcified apical thrombus. Further investigation by cardiac MRI revealed
apical thrombus and late apical uptake suggesting Endomyocardial
Fibrosis of possible eosinophilic etiology. The patient continued to
have attacks of similar chest pain for which stress cardiac MRI done and
was negative for ischemia. Other diagnostic work-up was done including
hematological and serological tests such as Antinuclear Antibodies and
Schistosoma Antibodies.

The patient was put on valsartan and Bisoprolol with oral anticoagulant
(vitamin K antagonist) and Rosuvastatin.

Conclusion: EMF may have heterogeneous presentation and should be
considered in patient with calcific apical thrombus without previous
history of cardiac problem, even in non-tropical region

\textbf{Introduction:}Endomyocardial fibrosis (EMF) is a progressive
restrictive cardiomyopathy that usually affects the left, right, or both
ventricles. It can also affect the outflow of the ventricles, leading to
the development of symptoms of failure of the involved ventricle (1).
The restrictive involvement is usually seen because of apical fibrosis
which usually arises from collagen deposition and fibroblast
proliferation. It is prevalent in tropical and subtropical parts of the
world. The main cause of it is still unknown, however, there are
multiple theories available regarding its development (2). Treatment is
challenging as currently there is limited data, medical therapy as a
case of restrictive heart failure might offer a symptomatic relief,
however the disease is usually progressive, and the long-term outcome is
generally poor. On the other hand, surgical resection of the endocardium
might offer a definite treatment however there is still a risk of
recurrence, and it carries a high mortality rate (3).

We report a patient who presented with chest pain and on subsequent
investigations were diagnosed with EMF. The patient has frequent visit
to Heart failure clinic, and he is doing well.

.

\textbf{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
(\textbf{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 \textbf{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, (\textbf{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 (\textbf{Figure 3} ) which demonstrated non-ischemic
intense LV apical sub-endocardial late gadolinium enhancement
(\textbf{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.

\textbf{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).





\textbf{Data availability statement:}The data that support the findings
of this study are available from the corresponding author upon
reasonable request.

\textbf{Conflict of interest:} The authors have no conflict of interest
to declare.

\textbf{Funding information}

Qatar national library -- Qatar foundation

\textbf{Ethical Approval}

The case report was approved by Hamad medical corporation, MRC number
MRC-04-21-242.

\textbf{Acknowledgments}

We thank the internal medicine department at Hamad Medical Corporation
for giving us the opportunity and support to conduct this work.

\textbf{Authors Contribution:} Dr Wafer dabdoob: Clinical care Dr ahmad
matarneh: Clinical care, literature review, and manuscript write up Dr
yousif alhailan: Clinical care, literature review, and manuscript write
up Dr sabir abdul karim: imaging Dr Maryam al kuwari : imaging

\textbf{References:}

\begin{enumerate}
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\end{enumerate}

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