DrKrishnaKanth G.
Nizam’s Institute of Medical Sciences,
Hyderabad.
The term 'endomyocardial disease' includes endomyocardial
fibrosis and Loffler's endocarditis parietalis fibroplastica
(L...
An association with eosinophils in the pathogenesis
of endomyocardial fibrosis was suggested by Davies
and Ball (1955).
...
Definition
Endomyocardial fibrosis (EMF) is an obliterative
cardiomyopathy of uncertain etiology, with fibrotic
deposits ...
Internationally: EMF occurs primarily in the subtropical
regions of Africa but is also encountered in tropical and
subtro...
EMF - India
First identified – Dr. Ball , CMC , Vellore
First publication – Samuel & Anklesaria
Gopi etal – described f...
Incidence
Africa – 10 -12 % of all heart disease
- MC cause of cardiomyopathy
- 3-25 % of autopsies
• India – south – 5-7...
EMF is primarily a disease of the young, occurring in
children, adolescents and young adults.
In Uganda, a bimodal peak ...
Ventricular chamber affected..
Africa – LV – 40%
RV – 10 %
Bi V – 50 %
• India - South ( vijayaraghavan etal ) –
RV – 60 ...
Etiology
Abraham , shaper , cadell et al – unusual expression
of rheumatic process
Vendergeld et al – autoimmunity- anti...
Etiology
EMF is most frequently observed in the socially disadvantaged and in
children and young women.
 These groups fr...
Etiology
Causes: A specific single etiology of EMF has not been
established. Suggested potential causes include the follo...
Pathophisiology
Edington & jackson – Basic lesion in heart muscle ,sec
changes in Endo/sub-endocardium
Davies & Ball – p...
Pathophysiology
In EMF, patchy fibrosis of the endocardial surface of
the heart, leads to reduced compliance and, ultimat...
Macroscopically, the hearts are hypertrophied and
the ventricular cavity may be dilated or reduced in
size.
The striking...
Pathophysiology 25
•The earliest changes of EMF are not well described
because most patients do not present with symptoms ...
Ultimately, after several years of disease activity, the
fibrotic phase is reached, when the endocardium is
replaced by c...
Figure 6. Histology of the LA demonstrates marked fibrotic thickening of the endocardium
(arrow), with proliferation of fi...
Typically, in right-sided involvement, the apex is
affected, gradually being drawn towards the tricuspid
valve, which may...
In 68% of patients, the posterior mitral leaflet is
involved and may be reduced to little more than a
fibrous ridge, perm...
Figure 5. At autopsy, the left ventricular apex was obliterated by prominent trabeculations
and fibrosis (mushroom sign; a...
Left Ventricle:
The region most commonly involved was the
posterior wall (29/30 cases), closely followed by the
apex and...
In the later stages of right ventricular disease, tricuspid
incompetence causes a very large "paper-thin" right
atrium, w...
Davies et.,al
Davies et.,al
Biventricular EMF
Histologic Findings: The heart size is not usually
enlarged in EMF.
The ventricular cavities are frequently laden with
t...
Hassan, W. M. et al. Chest 2005;128:3985-3992
Photomicrograph of endomyocardial biopsy specimen showing marked
thickening ...
Clinical features
Sex: Women of reproductive age and children are more
commonly affected than men.
Age:
EMF is not gene...
Clinical features
50
History: Typically, endomyocardial fibrosis (EMF) has an
insidious onset, and symptoms relate to the...
With left ventricular involvement, dyspnea is the
predominant symptom, especially exertional dyspnea.
Additionally, fatig...
Physical: Physical findings are also dependent on the extent
and distribution of disease.
In those with right ventricula...
Patients with Isolated RVEMF with severe tricuspid
regurgitation (TR) present with features of chronic right heart
failur...
Left-sided disease.
 Signs of pulmonary congestion are present in patients
with Signs of pulmonary hypertension and left...
Other Problems to be Considered:
Anthracycline toxicity
Carcinoid heart disease
Drug-induced cardiotoxicity (eg, serotoni...
Lab Studies:
Complete blood cell count may show anemia and
eosinophilia
Imaging Studies:
Chest radiography
The cardia...
Electrocardiography
Atrial fibrillation – more common in RVEMF than in
LVEMF
Low QRS voltage
First-degree AV block in u...
CXR
On chest radiographs,esp. in RVEMF the heart is always
enlarged in the transverse diameter, and often it is enormous....
In late cases there may be an oblique, linear calcification at the
elevated apex of the right ventricle or base of the pu...
RV EMF 60
The patterns of calcification in EMF. The top row shows
the typical linear oblique pattern of right-sided disease.
The low...
Echocardiography
Echocardiography is a useful tool when making the
diagnosis of EMF and has been demonstrated to
successf...
A pericardial effusion is frequently present and may
be large.
Diastolic function by Doppler echocardiography tend to
co...
Echocardiography
Features of RV EMF –
Dilated RVOT
Partially obliterated RV cavity/ inflow tract fibrosis
Thickened IV...
Echocardiography
Features of LV EMF –
Involvement of the posterior cusp and the posterior
papillary muscle (unlike in RH...
Hassan, W. M. et al. Chest 2005;128:3985-3992
Bidimensional echocardiographic apical two-chamber view of a patient with le...
LV EMF
LV EMF – apical fibrosis , PML
tethering 70
Electron beam computed
tomography scanning
Features of EMF observed with this modality were
described in the mid 1990s.
...
Cardiovascular magnetic
resonance imaging
Recently, the use of cardiovascular magnetic
resonance imaging has been shown t...
Steady-state free-precession 4-chamber view cine
MRI demonstrates right and left atrial dilatation and TR, with the
origin...
Steady-state free-precession cine MRI demonstrates
left atrial dilatation and mitral regurgitation (arrow).
Figure 4. Delayed-enhancement MRI of left ventricle radial view demonstrates
subendocardial hyperenhancement of the apex o...
Copyright © 2006 by the American Roentgen Ray Society
Salanitri, G. C. Am. J. Roentgenol. 2005;184:1432-1433
--59-year-old...
Copyright © 2006 by the American Roentgen Ray Society
Salanitri, G. C. Am. J. Roentgenol. 2005;184:1432-1433
--IV infusion...
Procedures:
Cardiac catheterization likely exhibits
hemodynamic findings consistent with restrictive
cardiomyopathy.
Fi...
Hassan, W. M. et al. Chest 2005;128:3985-3992
Right-heart pressure tracing in a patient with right-sided EMF showing incre...
Hassan, W. M. et al. Chest 2005;128:3985-3992
Right and left heart pressure tracing in a patient with biventricular EMF sh...
Vijaya Raghavan
Angiography
Traditionally, angiography has been considered the
criterion standard when making the diagnosis of EMF.
Left...
Angiocardiography
Features of RV EMF –
1) Aneurysmal dilatation of RA
2)Small and fibrosed inflow tract of RV
3)Dilated ,...
Angiocardiography
Features of LV EMF –
1) Irregular outline of endocardium
2)Filling defects
3)Mitral regurgitation
4)Red...
Angiocardiography
RV EMF – Grading
Grade 1 – Minimal involvement of RV chamber in the form
of alterations in the trabecu...
Angiocardiography
LV EMF – Grading –
Grade 1 – Generalised smoothening of the LV wall
with small irregular filling defec...
Hassan, W. M. et al. Chest 2005;128:3985-3992
Left ventricular angiogram in the right anterior oblique view showing oblite...
Hassan, W. M. et al. Chest 2005;128:3985-3992
Right ventricular angiogram in the right anterior oblique view in a patient ...
Clenched Fist appearance
BI V EMF
LV EMF - MR
RV EMF – dilated RA
RV EMF – dilated pulm conus
RV EMF –aneurysmal RA
Diagnosis
 Routine radiography will be of great
assistance in right-sided dominance, but
echocardiography and angiocardio...
Panel A. Fibrotic left ventricle (LV) apical infiltration (arrow).
LA, left atrium.
Panel B. Left ventriculography showing...
Prognosis :
Prognosis for this condition is poor.
Incidence of sudden cardiac death from fatal
arrhythmias or from progr...
Treatment
Medical Care:
In general, the response to medical therapy is poor and
unproven.
Because most patients with en...
For patients with severe symptoms, consider surgical
therapy because the prognosis for these patients with
continued medi...
Treatment
Surgical Care:
Surgical therapy by endocardial decortication seems to
be beneficial for many patients with adv...
In 1971, Dubost et al.41 had introduced surgical
treatment of EMF by endocardial decoritication and
AV valve replacement....
BDG shunt is offered only to patients with isolated
RVEMF, with no pulmonary hypertension, mitral
incompetence or diastol...
 Because the myocardium is not usually affected, the severe hemodynamic
derangement associated with EMF is relieved with ...
The rationale for surgery has been the following.
 Poor prognosis in the long term with medical treatment
in class III a...
LV apical thrombus
Lepley first operated successfully on advanced EMF by
performing endocardial decortication and mitral valve
replacement (...
Keys to long-term success include thorough
decortication and bioprosthetic valve replacement :
xenografts constitute the ...
Mitral valve repair (MVr) has been described for
advanced EMF but not in the acute stages
Early postoperative recurrence...
Hassan, W. M. et al. Chest 2005;128:3985-3992
Left ventricular angiogram in the right anterior oblique view showing a smal...
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Emf final present
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  • Figure 6. Histology of the LA demonstrates marked fibrotic thickening of the endocardium (arrow), with proliferation of fibrous tissue in the underlying myocardium, which is consistent with endomyocardial fibrosis (Masson trichrome stain, original magnification ×50).
  • Figure 5. At autopsy, the left ventricular apex was obliterated by prominent trabeculations and fibrosis (mushroom sign; arrows).
  • Figure 4. Delayed-enhancement MRI of left ventricle radial view demonstrates subendocardial hyperenhancement of the apex of the left ventricle, suggesting fibrosis (arrows). Abbreviations as in Figure 2.
  • Emf final present

    1. 1. DrKrishnaKanth G. Nizam’s Institute of Medical Sciences, Hyderabad.
    2. 2. The term 'endomyocardial disease' includes endomyocardial fibrosis and Loffler's endocarditis parietalis fibroplastica (Loffler's endomyocardial disease)which have long been considered separate entities. Endomyocardial fibrosis was believed to be confined to the tropical regions, and Loffler's endomyocardial disease to temperate zones and associated with eosinophilia The geographical limitations of the tropical type of endomyocardial fibrosis, suggesting confinement to the African continent, has proved unwarranted .
    3. 3. An association with eosinophils in the pathogenesis of endomyocardial fibrosis was suggested by Davies and Ball (1955). Subsequently, Parry and Abrahams (1965) noted patients in Nigeria, either diagnosed as Loffler's disease or heart muscle disease due to filariasis. Europeans, working in the tropical zones who developed endomyocardial fibrosis in association with eosinophils, were described by Brockington, Olsen and Goodwin (1967).
    4. 4. Definition Endomyocardial fibrosis (EMF) is an obliterative cardiomyopathy of uncertain etiology, with fibrotic deposits on the endocardial surface of the apices and inflow of either or both ventricles. It is a progressive cardiac disease characterized by swelling of the endocardial connective tissue, with accumulation of acid mucopolysaccharides in the endocardium followed by scarring and fibrosis. It may affect one or both ventricles primarily, and the other cardiac chambers to a lesser extent,not accompanied by lesions in any other part of the body (except when there is embolization).
    5. 5. Internationally: EMF occurs primarily in the subtropical regions of Africa but is also encountered in tropical and subtropical regions elsewhere in the world, including areas in India and South America that are within 15° of the equator. More than 90% of reported cases of EMF have occurred in geographic locations that are within 15° of the equator. In equatorial African nations, such as Nigeria, EMF is the fourth most common cause of cardiac disease in adults, and EMF accounts for 22% of cases of heart failure in Nigerian children. EMF is the most common type of restrictive cardiomyopathy in tropical countries
    6. 6. EMF - India First identified – Dr. Ball , CMC , Vellore First publication – Samuel & Anklesaria Gopi etal – described features of RV EMF Vijayaraghavan etal , cherian etal – described the clinical , radiological , hemodynamic and angiographic features of LV/RV EMF in south India
    7. 7. Incidence Africa – 10 -12 % of all heart disease - MC cause of cardiomyopathy - 3-25 % of autopsies • India – south – 5-7 % of all admissions ( Nair etal) - North - < 5 % of all admissions - 0.75 % of all autopsies ( wahi etal)
    8. 8. EMF is primarily a disease of the young, occurring in children, adolescents and young adults. In Uganda, a bimodal peak at ages 10 and 30 has been observed, and a similar pattern was recently found in Mozambique. The differences between genders in the frequency of disease have been variable . ages varied from 7 to 31 years. (vijaya raghavan et .,al)
    9. 9. Ventricular chamber affected.. Africa – LV – 40% RV – 10 % Bi V – 50 % • India - South ( vijayaraghavan etal ) – RV – 60 % LV – 20 % BiV – 20 % - North (wahi etal ) – RV – 20 % LV – 40 % Bi V – 40 %
    10. 10. Etiology Abraham , shaper , cadell et al – unusual expression of rheumatic process Vendergeld et al – autoimmunity- antiheart ab Connor et al – multifactorial hypothesis Seyle et al – malnutrition , Mg , K loss Crawford etal –ingestion of serotonin rich food Grey et al,miller et al – Loa loa infection Smith & furth – chronic beriberi
    11. 11. Etiology EMF is most frequently observed in the socially disadvantaged and in children and young women.  These groups frequently have malnutrition, and in regions of sub- Saharan Africa where the disease is most prevalent, the typical diet is high in a tuber called cassava, which contains relatively high concentrations of the rare earth element cerium (Ce).  The combination of high Ce levels and hypomagnesemia produced EMF-like lesions in laboratory animals. A familial tendency has rarely been noted in Uganda and Zambia
    12. 12. Etiology Causes: A specific single etiology of EMF has not been established. Suggested potential causes include the following: Infectious causes Parasites (eg, helminths) Protozoans (eg, toxoplasmosis, malaria) Inflammatory causes - Eosinophilia Nutritional causes General malnutrition High-tuber diet Ce toxicity Hypomagnesemia
    13. 13. Pathophisiology Edington & jackson – Basic lesion in heart muscle ,sec changes in Endo/sub-endocardium Davies & Ball – predominant endocardial involvement Mehrotra etal – degeneration of myocardium Kinare & deshpande – interstitial fibrosis & atrophy of the myocardium Samuel etal – myocardial degeneration Reddy etal - myocytolysis
    14. 14. Pathophysiology In EMF, patchy fibrosis of the endocardial surface of the heart, leads to reduced compliance and, ultimately, restrictive physiology as the endomyocardial surface becomes more generally involved.  Endocardial fibrosis principally involves the inflow tracts of the right and left ventricles and may affect the atrioventricular valves, leading to tricuspid and mitral regurgitation.
    15. 15. Macroscopically, the hearts are hypertrophied and the ventricular cavity may be dilated or reduced in size. The striking feature is the immense thickening of the endocardium, often several millimeters in dimension. Strands of fibrous tissue frequently extend into the underlying myocardium, usually, limited to the inner third of the myocardial wall (Davies and Ball, 1955; Olsen, 1972). The right ventricle (11%), the left ventricle (38%) or both ventricles (51%) may be involved (Shaper, Hutt and Coles, 1968).
    16. 16. Pathophysiology 25 •The earliest changes of EMF are not well described because most patients do not present with symptoms until relatively late in the clinical course. o Olsen described 3 phases of EMF. •The first phase involves eosinophilic infiltration of the myocardium with necrosis of the subendocardium and a pathologic picture consistent with acute myocarditis. This is reportedly present in the first 5 weeks of the illness. The second stage, typically observed after 10 months, is associated with thrombus formation over the initial lesions, with a decrement in the amount of inflammatory activity present.
    17. 17. Ultimately, after several years of disease activity, the fibrotic phase is reached, when the endocardium is replaced by collagenous fibrosis. Extensive calcification is rarely associated with fibrosis. This pathomorphologic scheme is not observed uniformly and has not been consistently supported by other investigators.
    18. 18. Figure 6. Histology of the LA demonstrates marked fibrotic thickening of the endocardium (arrow), with proliferation of fibrous tissue in the underlying myocardium, which is consistent with endomyocardial fibrosis (Masson trichrome stain, original magnification ×50). Cury R C et al. Circulation 2005;111:e115-e117 Copyright © American Heart Association
    19. 19. Typically, in right-sided involvement, the apex is affected, gradually being drawn towards the tricuspid valve, which may also be affected by this process Thus, the cavity is progressively obliterated. The chordae tendineae and papillary muscles may also be involved. In left ventricular involvement, the inflow tract, apex and part of the outflow tract is usually affected. (Olsen) The thick endocardium ends, usually abruptly, in a rolled edge in the region beneath the anterior mitral valve leaflet (Davies)
    20. 20. In 68% of patients, the posterior mitral leaflet is involved and may be reduced to little more than a fibrous ridge, permitting mitral regurgitation which does not always produce the typical murmur on auscultation The valve leaflets otherwise remain intrinsically normal unless there is bacterial or rheumatic infection. Gradually, obliteration of the ventricular apex occurs which, with cicatrization, reduces the volume and alters the shape of the affected ventricular chamber
    21. 21. Figure 5. At autopsy, the left ventricular apex was obliterated by prominent trabeculations and fibrosis (mushroom sign; arrows). Cury R C et al. Circulation 2005;111:e115-e117 Copyright © American Heart Association
    22. 22. Left Ventricle: The region most commonly involved was the posterior wall (29/30 cases), closely followed by the apex and lateral wall (both 25/30 cases); the septal wall was less frequently affected (15/30)(Davies et.,al)
    23. 23. In the later stages of right ventricular disease, tricuspid incompetence causes a very large "paper-thin" right atrium, with massive thrombi in the atrial appendage.  These sometimes extend into the superior vena cava or the veins draining into it. The right ventricle becomes contracted and distorted, with a hypertrophied, dilated outflow tract. In left-sided involvement, the left atrium is enlarged, unless partially protected by the low flow rates of biventricular disease. The right coronary artery is displaced by the bulging of the atrioventricular groove, but the coronary lumen is not compromised on either side.
    24. 24. Davies et.,al
    25. 25. Davies et.,al
    26. 26. Biventricular EMF
    27. 27. Histologic Findings: The heart size is not usually enlarged in EMF. The ventricular cavities are frequently laden with thrombi and, in severe cases, may be nearly totally obliterated by endocardial thickening and thrombosis. The histologic findings are characterized by reactive fibrosis with a selective increase in type I collagen deposition, subendocardial infarction and fibrosis, and thrombus formation. Additionally, specific features of other diseases, such as those associated with hemochromatosis or
    28. 28. Hassan, W. M. et al. Chest 2005;128:3985-3992 Photomicrograph of endomyocardial biopsy specimen showing marked thickening of the endocardium (E) with fibrosis (hematoxylin-eosin, original x 200)
    29. 29. Clinical features Sex: Women of reproductive age and children are more commonly affected than men. Age: EMF is not generally observed in children younger than 4 years, although the typical pathology for EMF has recently been described in a 4-month-old infant with left ventricular inflow tract obstruction. The people most commonly affected are older children (aged 5-15 y) and young adults, but cases have been reported in individuals aged 70 years.
    30. 30. Clinical features 50 History: Typically, endomyocardial fibrosis (EMF) has an insidious onset, and symptoms relate to the specific chambers and valves where the disease is most extensive. The triad of raised jugular venous pressure, hepatomegaly, and ascites characterize of right ventricular endomyocardial fibrosis (VijayaRaghavan et.,al) When right ventricular involvement or tricuspid regurgitation predominates, lower extremity swelling, increasing abdominal girth, and nausea may be expected.
    31. 31. With left ventricular involvement, dyspnea is the predominant symptom, especially exertional dyspnea. Additionally, fatigue, paroxysmal nocturnal dyspnea, and orthopnea may be present. Thromboembolic complications may occur in EMF. Rarely, patients may present early in the course of the disease with an acute febrile illness with symptoms of cardiac insufficiency mimicking myocarditis. Recently, angina like chest pain and syncope were reported in a patient with EMF involving the left ventricle
    32. 32. Physical: Physical findings are also dependent on the extent and distribution of disease. In those with right ventricular involvement, jugular venous pressure elevation, ascites, and edema may be present. The presence of ascites may appear out of proportion to the amount of peripheral edema. Patients with tricuspid regurgitation may have giant V waves. A S3 or S4 and tachycardia may be present.
    33. 33. Patients with Isolated RVEMF with severe tricuspid regurgitation (TR) present with features of chronic right heart failure with markedly elevated JVP and expansile large ‘v’ waves, pulsatile liver, hepatomegaly, ascites, oedema, cyanosis, cachexia and malnutrition. They may also have pericardial effusion, marked cardiomegaly, RV S3, and inconspicuous systolic murmur of TR. The severity of TR rather than the presence of RV diastolic dysfunction is the more important determining factor for the clinical outcome of patients An occasional patient with severe RVEMF can have cyanosis due to right-to-left atrial shunting through a patent and stretched fossa ovalis defect.
    34. 34. Left-sided disease.  Signs of pulmonary congestion are present in patients with Signs of pulmonary hypertension and left heart failure are out of proportion to the degree of MR Isolated LVEMF, in the absence of AV valve incompetence, is often minimally symptomatic. Hemodynamic study may reveal a prominent ‘a’ wave in the PA wedge pressure, and LV end diastolic pressure may be elevated.
    35. 35. Other Problems to be Considered: Anthracycline toxicity Carcinoid heart disease Drug-induced cardiotoxicity (eg, serotonin, methysergide, ergotamine, mercurial agents, busulfan) Fabry disease Fatty infiltration Gaucher disease Glycogen storage disease Hurler disease Idiopathic cardiomyopathy Metastatic cancers Radiation Rheumatic heart disease Occasionally, a masslike lesion seen in endomyocardial fibrosis masquerades as an intracardiac tumor.
    36. 36. Lab Studies: Complete blood cell count may show anemia and eosinophilia Imaging Studies: Chest radiography The cardiac silhouette in endomyocardial fibrosis (EMF) may be normal in size, and generalized cardiomegaly is unusual Significant enlargement of the atria, and significant right atrial enlargement creates a cardiac silhouette in the shape of the African continent, (‘Heart of Africa.’)
    37. 37. Electrocardiography Atrial fibrillation – more common in RVEMF than in LVEMF Low QRS voltage First-degree AV block in up to 44% of patients Incomplete RBBB in up to 30% of patients IN RV EMF – ‘p’ pulmonale , RVH , QR pattern in V 1 , Q in inf leads In LVEMF – ‘p’ mitrale , LVH
    38. 38. CXR On chest radiographs,esp. in RVEMF the heart is always enlarged in the transverse diameter, and often it is enormous. This may be due to a coexisting pericardial effusion, but is usually due to a dilated, almost aneurysmal right atrium.  If pericardial fluid is scanty there will be an outflow tract convexity, which on fluoroscopy or ultrasound is seen to be very active.
    39. 39. In late cases there may be an oblique, linear calcification at the elevated apex of the right ventricle or base of the pulmonary conus .  The lung fields are strikingly oligemic and, because of low cardiac output, the superior vena cava and azygos veins are very prominent. In LV EMF , myocardial calcification and pulmonary congestion may be seen with a moderate cardiomegaly
    40. 40. RV EMF 60
    41. 41. The patterns of calcification in EMF. The top row shows the typical linear oblique pattern of right-sided disease. The lower row displays the curvilinear left ventricular form.
    42. 42. Echocardiography Echocardiography is a useful tool when making the diagnosis of EMF and has been demonstrated to successfully differentiate EMF and other processes. The presence and location of fibrosis as determined by echocardiography correlates well with autopsy findings. Findings include thickening of the inferior and basal left ventricular wall, apical obliteration, thrombi adherent to endocardial surface, mitral regurgitation, and tricuspid regurgitation.
    43. 43. A pericardial effusion is frequently present and may be large. Diastolic function by Doppler echocardiography tend to correlate with the functional status of the patient. most patients present with later stages of EMF, a restrictive filling pattern in the left ventricular outflow tract is most common. Recently, decreased flow propagation velocity (Vp) Color-flow imaging frequently exhibits tricuspid and mitral regurgitation. Spectral Doppler analysis of tricuspid regurgitation frequently reflects an increased pulmonary artery systolic pressure.
    44. 44. Echocardiography Features of RV EMF – Dilated RVOT Partially obliterated RV cavity/ inflow tract fibrosis Thickened IVS near the apical region Hugely dilated RA ‘Notch’ on the epicardial surface near the apex of the RV – occurs in advanced disease Pericardial effusion Restriction of movement of tricuspid valve cusps
    45. 45. Echocardiography Features of LV EMF – Involvement of the posterior cusp and the posterior papillary muscle (unlike in RHD , AML is spared) Obliteration of LV apex Presence of areas of dyskinesia and aneurysmal dilatation
    46. 46. Hassan, W. M. et al. Chest 2005;128:3985-3992 Bidimensional echocardiographic apical two-chamber view of a patient with left- sided EMF (top, a) and a four-chamber view of a patient with right-sided EMF (bottom, b)
    47. 47. LV EMF
    48. 48. LV EMF – apical fibrosis , PML tethering 70
    49. 49. Electron beam computed tomography scanning Features of EMF observed with this modality were described in the mid 1990s. The fibrotic process is delineated as a band of low attenuation within the endocardium. Obliteration of the apex and inflow tract, when present, is also demonstrated. This method reportedly assists in distinguishing EMF from constrictive pericarditis.
    50. 50. Cardiovascular magnetic resonance imaging Recently, the use of cardiovascular magnetic resonance imaging has been shown to demonstrate obliterative changes in the ventricles, atrial dilation, and regurgitant atrioventricular valve lesions in patients with EMF. However, the use of contrast-enhanced imaging was not able to demonstrate fibrosis within the ventricles of these patients.
    51. 51. Steady-state free-precession 4-chamber view cine MRI demonstrates right and left atrial dilatation and TR, with the origin of the TR jet dislocated toward the apex of the right ventricle (RV; arrow), possibly secondary to papillary muscle fibrosis
    52. 52. Steady-state free-precession cine MRI demonstrates left atrial dilatation and mitral regurgitation (arrow).
    53. 53. Figure 4. Delayed-enhancement MRI of left ventricle radial view demonstrates subendocardial hyperenhancement of the apex of the left ventricle, suggesting fibrosis (arrows). Cury R C et al. Circulation 2005;111:e115-e117 Copyright © American Heart Association
    54. 54. Copyright © 2006 by the American Roentgen Ray Society Salanitri, G. C. Am. J. Roentgenol. 2005;184:1432-1433 --59-year-old woman with endomyocardial fibrosis and intracardiac thrombus HASTE image showing high signal intensity in endocardium of both ventricles
    55. 55. Copyright © 2006 by the American Roentgen Ray Society Salanitri, G. C. Am. J. Roentgenol. 2005;184:1432-1433 --IV infusion of double-dose gadolinium shows intense hyperenhancement of right and left ventricular endocardium (arrows), an appearance consistent with fibrosis. Overlying thrombus has very low signal intensity on this sequence.
    56. 56. Procedures: Cardiac catheterization likely exhibits hemodynamic findings consistent with restrictive cardiomyopathy. Findings from endomyocardial biopsy may be diagnostic, but this procedure is typically not needed. Biopsy findings may be nondiagnostic when the disease is patchy and sampling sites do not correlate with areas of disease. Because biopsy (especially from the left ventricle) carries some risk, reserve the use of
    57. 57. Hassan, W. M. et al. Chest 2005;128:3985-3992 Right-heart pressure tracing in a patient with right-sided EMF showing increased right atrial pressure with a prominent A wave that is seen also in the right ventricular and pulmonary artery (PA) tracings
    58. 58. Hassan, W. M. et al. Chest 2005;128:3985-3992 Right and left heart pressure tracing in a patient with biventricular EMF showing elevated RA, RV, pulmonary artery (PA), left ventricular end-diastolic pressure, and pulmonary capillary (P) wedge pressures
    59. 59. Vijaya Raghavan
    60. 60. Angiography Traditionally, angiography has been considered the criterion standard when making the diagnosis of EMF. Left and right ventriculography exhibits distortion of chamber morphology by fibrosis and obliteration and variable degrees of mitral and tricuspid regurgitation. The mushroom sign has been used to describe the shape of the affected ventricle when the apex is obliterated completely by fibrosis.
    61. 61. Angiocardiography Features of RV EMF – 1) Aneurysmal dilatation of RA 2)Small and fibrosed inflow tract of RV 3)Dilated , hyperdynamic outflow region 4)Normally placed tricuspid valve
    62. 62. Angiocardiography Features of LV EMF – 1) Irregular outline of endocardium 2)Filling defects 3)Mitral regurgitation 4)Reduced EF
    63. 63. Angiocardiography RV EMF – Grading Grade 1 – Minimal involvement of RV chamber in the form of alterations in the trabecular pattern at the apex and along the septal border with irregular filling defects Grade 2 – Obliteration of the apex and adjacent border of the RV chamber ,but not extending up to the tricuspid annulus Grade 3 – Obliteration of the RV chamber including the area near the tricuspid annulus ,but sparing the RV outflow Grade 4 – Involvement of the RV body as well as narrowing of the outflow tract
    64. 64. Angiocardiography LV EMF – Grading – Grade 1 – Generalised smoothening of the LV wall with small irregular filling defects at the apex Grade 2 – Obliteration of the apical area of the LV chamber in addition to a smooth border and irregular filling defects Grade 3 – Obliteration of roughly half or more of the LV cavity
    65. 65. Hassan, W. M. et al. Chest 2005;128:3985-3992 Left ventricular angiogram in the right anterior oblique view showing obliteration of the apex (arrow) in systole (top, a) and diastole (bottom, b)
    66. 66. Hassan, W. M. et al. Chest 2005;128:3985-3992 Right ventricular angiogram in the right anterior oblique view in a patient with right-sided EMF showing complete obliteration of the apex of the right ventricle, dilated right atrium, and severe TR
    67. 67. Clenched Fist appearance
    68. 68. BI V EMF
    69. 69. LV EMF - MR
    70. 70. RV EMF – dilated RA
    71. 71. RV EMF – dilated pulm conus
    72. 72. RV EMF –aneurysmal RA
    73. 73. Diagnosis  Routine radiography will be of great assistance in right-sided dominance, but echocardiography and angiocardiography are important, particularly in distinguishing left- sided EMF from rheumatic disease.  MRI may replace angiography except in some preoperative patients.
    74. 74. Panel A. Fibrotic left ventricle (LV) apical infiltration (arrow). LA, left atrium. Panel B. Left ventriculography showing the amputated LV apex (arrow). Panel C. TV (white arrow); diffuse RV fibrotic infiltration (black arrow). Panel D. Contrast medium in the superior cava vein (black arrow) descending through the right pulmonary artery (white arrow
    75. 75. Prognosis : Prognosis for this condition is poor. Incidence of sudden cardiac death from fatal arrhythmias or from progressive cardiac failure is high. Most patients have extensive disease at the time of presentation; therefore, survival after diagnosis is relatively brief. In one study, 95% of a group of patients had died at 2 years. In a second study, 44% of patients died within 1 year after the onset of symptoms.
    76. 76. Treatment Medical Care: In general, the response to medical therapy is poor and unproven. Because most patients with endomyocardial fibrosis (EMF) present long after any possible period of early active myocarditis may have existed, little role exists for immunosuppressive therapy . Symptomatic therapy with diuretics has been shown to be useful, but digoxin, afterload reducers, and beta-blockers have little role in EMF.
    77. 77. For patients with severe symptoms, consider surgical therapy because the prognosis for these patients with continued medical therapy alone is dismal. Because the rate of thromboembolism is low among patients with EMF The patient population affected is not typically compliant with anticoagulation regimens. most authors do not recommend anticoagulant therapy.
    78. 78. Treatment Surgical Care: Surgical therapy by endocardial decortication seems to be beneficial for many patients with advanced disease who are in functional-therapeutic class III or IV. The operative mortality rate is high (15-20%), but successful surgery has a clear benefit on symptoms and seems to favorably affect survival as well. The most commonly used approach is endocardiectomy, combined with mitral and/or tricuspid repair or replacement (when indicated), using a midline thoracotomy and cardiopulmonary bypass.
    79. 79. In 1971, Dubost et al.41 had introduced surgical treatment of EMF by endocardial decoritication and AV valve replacement. A plane of cleavage can be easily developed and all of the yellow–white thickened endocardium removed. Surgical options are LV endocardiectomy, AV valve repair or replacement Exclusion of fibrotic RV in a pure RVEMF by a BDG connection (JaganMohan Tharakan SCTIMST et.,al)
    80. 80. BDG shunt is offered only to patients with isolated RVEMF, with no pulmonary hypertension, mitral incompetence or diastolic dysfunction. Any grade of LVEMF is considered a contraindication for BDG shunt.
    81. 81.  Because the myocardium is not usually affected, the severe hemodynamic derangement associated with EMF is relieved with successful resection of the endocardium.  Depending on the location of the disease (right or left ventricle, apex or inflow tract), a transapical or transventricular approach can be used.  Common postoperative complications include low cardiac output, heart block, and ventricular arrhythmias.
    82. 82. The rationale for surgery has been the following.  Poor prognosis in the long term with medical treatment in class III and IV patients (surgery offers 5 year survival of 65– 75%; 10 year survival10 of 60–70% Hemodynamic derangement is due to restriction and AV valve incompetence (correctable by endocardiectomy and AV valve repair or replacement). Rarity of myocardial involvement by fibrosis. Rarity of recurrence of same fibrotic process after endocardiectomy.
    83. 83. LV apical thrombus
    84. 84. Lepley first operated successfully on advanced EMF by performing endocardial decortication and mitral valve replacement (MVR) through an apical ventriculotomy . This has subsequently been adapted effectively for right sided disease. With 5-year survival of 72% achievable even amongst end-stage sufferers, surgery represents the only hope of prolonging survival in this incurable disease .
    85. 85. Keys to long-term success include thorough decortication and bioprosthetic valve replacement : xenografts constitute the bulk of reported experience whilst death from late thrombosis of a mechanical prosthesis has occurred Despite considerable surgical experience with managing the chronic fibrotic stages in LEM, the clinicopathological behaviour and perioperative course in the acute thrombotic stage remains a mystery.
    86. 86. Mitral valve repair (MVr) has been described for advanced EMF but not in the acute stages Early postoperative recurrence of suggests that MVR may be more appropriate. Rapid disease recurrence jeopardizing a conserved mitral valve in acute eosinophilic endomyocarditis cautions against surgical repair despite its many advantages. A bioprosthesis is associated with reduced thrombotic complications and may be the treatment of choice for this rare pathology.
    87. 87. Hassan, W. M. et al. Chest 2005;128:3985-3992 Left ventricular angiogram in the right anterior oblique view showing a small left ventricle with apical obliteration, severe MR, and dilated left atrium in a patient with left-sided EMF (top, a) and after surgery with endocardectomy and mitral valve replacement (bottom, b)
    88. 88. THANK YOU

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