Dilated cardiomyopathy is characterized by the slow, progressive dilation of all four heart chambers and impaired systolic function. It can occur at any age but is most common between 20-60 years old. The cause is often unknown but may be related to alcohol toxicity, pregnancy, genetic defects, heavy metal ingestion or cytotoxic drugs. Grossly, the heart is enlarged and flabby with poor contractility that can lead to thrombus formation. Microscopically, features include myocyte nuclear enlargement, reduced myocyte width, loss of myofibrils, interstitial fibrosis, and increased lymphocytes.

2.  Pericarditis : classification-
eitiology/morphology
 Cardiomyopathy:Types-
Dilated/Hypertrophied/Restricted
 Myocarditis: causes
 Cardiac tumors

5.  Fibrous sac surrounding heart-dense network
of collagen fibres
 Serous membrane – two continuous layers
separated by a small amount of fluid lubricant
(10-20mls straw coloured)
 Layers are called visceral and parietal
 Visceral is inner layer (epicardium)
 Parietal is continuous with diaphragm and outer
walls of great arteries

6.  Surrounds the heart
 Continuous with the great arteries and the
diaphragm

7.  Usually secondary to disorders involving the
heart, or adjacent mediastinal structures (Eg:
Myocardial infarct, trauma, radiation, tumour
or infections).
 Rarely, may be due to systemic abnormalities
(uremia, diabetes mellitus, chronic nephritis,
severe anemia , autoimmune disease).

8.  Viral Infections
 Purulent Pericarditis
 TB
 Mediastinal radiation
 MI
 Cardiac surgery
 Trauma
 Cardiac procedures
 Drugs andToxins
 Metabolic disorders
 Malignancies (breast,
lung, Hodgkin’s,
mesothelioma)
 CollagenVascular Disease
 Idiopathic

9.  Neoplastic-35%
 Immune Mediated- 23%
 Viral- 21%
 Bacterial-6%
 Uremia-6%
 TB- 4%
 Idiopathic-4%

10.  Common bugs
 Cocksackie A and B
 Echovirus
 Adenovirus
 Viral infections uncommon in patients
presenting with pericardial effusion w/o
pericarditis
 Exception is HIV- frequently presents with
significant effusion w/o pericarditis
 seen in 7 % of patients hospitalized with effusions

11.  Staphylococcus
 Pneumococccus
 Streptococcus(rheumatic pancarditis)
 Haemophilus
 M.Tuberculosis
 Can occur as systemic spread or direct extension
 Frequently purulent

12.  Histoplasma- most common fungus in
immunocompetent patients
 Especially the Ohio RiverValley
 In immunocompromised
 Aspergillus
 Candida
 Coccidoides
 Frequently purulent

13.  Rickettsia Ricketsii
 Chlamydia Psittaci
 Borrelia burgdorferi
 Treponema Pallidum
 Actinomycosis
 Mycoplasma Pneumonia
 Nocardia

14.  Pericardial involvement is related to infarct size
 Early stage - inflammatory etiology
 Late stage
 Immune mediated weeks to months out
 Known as Post Cardiac Injury syndrome (PCIS) or
Dressler’s syndrome
 Rare in modern time due to reperfusion therapies

15.  Mediastinal Radiation-wide spectrum of
diseases seen
 Cardiac Surgeries
 Cardiac Procedures
 Traumatic

16.  Lupus like sydromes
 Procainamide
 Hydralazine
 Phenytoin
 INH
 Penicillins- Hypersensitivity Pericarditis
 Chemotherapy
 Doxorubicin/Daunorubicin-cardiomyopathy/pericardiopathy
 Bleomycin - sclerosing agent

17.  Asbestosis can cause pericardial lesions
 Scorpion fish venom can cause pericarditis

18.  Uremia-
 Most common metabolic cause
 6-10 % of ESRD patients not on HD can have Pericarditis
 Dialysis related Pericardial Effusions (seen in 13% of
patients)
 Severe Hypothyroidism
 effusion – usually not significant
 rarely pericarditis
 Ovarian hyperstimulation syndrome
 complication of gonadotropin therapy
 Due to fluid shifts

19.  Responsible for 6% of acute pericardial disease
(pericarditis and tamponade)
 Accounts for 15-20% of moderate to large pleural
effusions
 Mets - Lung, Breast, Hodgkin’s metastases
 Primary - Mesotheliomas and lipomas

20.  SLE- pericardial involvement in up to 50%
 Rheumatoid Arthritis
 Progressive Systemic Sclerosis
 MCTD
 Polyarteritis
 Giant Cell Arteritis
 Inflammatory Bowel Disease

21.  In two large series (331 patients), only 16 %
had an identifiable cause of pericarditis
 Many of these cases are presumed viral
 Only 7-29% of patients have idiopathic
pericardial effusions

22.  Chest Pain-
 sudden onset over anterior chest
 sharp and pleuritic
 Improves by leaning forward
 Radiates commonly to trapezius ridges
 Pericardial Friction Rub
 ECG – findings depend on stage
 2 of 3 needed to make diagnosis +/- effusion.

23.  Classification on the basis of etiological factors:
 1. Acute nonspecific (idiopathic)
 2. Infective :
 a) Bacterial b)Viral c) Other infections
 3. Immunologic :
 a) Rheumatic fever b) Other connective tissue
disorder
 4. Neoplastic
 5. Metabolic :
 a) Uremic b) Myxedema c) Gout.
 6.Traumatic (including after cardiac surgery)
 7. Associated with myocardial infarction.

25.  Acute pericarditis - most often of viral origin.
 Acute pericarditis - heal by resolution or by
pericardial fibrosis ranging from a thick,
pearly, nonadherent epicardial plaque , to
thin delicate adhesion to massive adhesions.

26. 26

27.  Morphological Classification of acute
pericarditis:
 SEROUS: SLE, scleroderma, tumors, uremia
 FIBRINOUS: MI (Dressler), uremia, radiation, RF,
SLE, s/p open heart surgery
 PURULENT: infective, bacterial
 HEMORRHAGIC: Malignancy,TB
 CASEOUS:TB
 CHRONIC: (ADHESIVE, CONSTRICTIVE)

28.  Serous pericarditis :
 Usually consists of 50 to 200 ml of slowly
accumulating exudates
 Characteristically produced by nonbacterial
involvement, including rheumatic fever,
systemic lupus erythematosus, tumours, uremia
and primary viral infection (Eg. Coxsackie)
 Microscopically- scant epicardial or pericardial
acute and chronic inflammatory infiltrates.

30.  Fibrinous and serofibrinous pericarditis:
 Most common clinical form, seen in myocardial
infarct with a pericardial friction rub.
 Causes-Acute MI,Dressler’s syn,uraemia,chest
radiation,RF,SLE,rheumatoid heart disease,
trauma and routine cardiac surgery.
 Morphology-maybe dry or yellow,thick and
cloudy fluid
 Loud pericardial friction rub
 Exudate may be completely resolved or be
organized causing adhesive pericarditis.

31. Bread and butter
pericarditis

32.  Purulent (suppurative) pericarditis:
 Due to bacteria, fungus or parasitic infection.
 Infection reaches by direct extension, by
hematogenous or lymphatic route from
neighbouring areas of infection e.g.
Pneumonia, empyema, lung abscess,
subphrenic abscess, liver abscess etc or
during cardiotomy.

33.  Most common causative organisms-
Staphylococci, Streptococci, and Pneumococci.
 Composed of 400 to 500 ml of a thin to creamy
pus with erythematous, granular serous
surfaces.
 Patient presents with fever, rigor and a friction
rub.
 It usually organizes and may produce
mediastinopericarditis or constrictive
pericarditis.

35.  Hemorrhagic pericarditis:
 Composed of exudates of blood admixed
with fibrinous to suppurative effusion.
 Most commonly it follows cardiac surgery or
is associated with tuberculosis or malignancy.
 It usually organizes with or without
calcification.

37.  Caseous pericarditis:
 Due to tuberculosis (by direct extension from
neighbouring lymphnodes) or less commonly,
mycotic infection.
 This type most frequently, causes fibrocalcific
constrictive pericarditis

39.  Healed stage of pericardial inflammation.
 Seen in tuberculosis and fungal infections, which on
healing may lead to damaging adhesion.
 In some cases, organization merely produces thick,
pearly, nonadherent epicardial plaque , to thin
delicate adhesion of obscure origin.

40.  Chronic pericarditis may be of the following
types:
 1.Adhesive pericarditis :
 Chronic pericarditis with adhesion between
parietal and visceral pericardium.
 Mostly seen in rheumatic disease.
 Less commonly, may be due to infection by
pyogenic bacteria, tuberculosis etc.
 In some cases, the cause is unknown.

41.  2.Adhesive mediastinopericarditis:
 Pericardial sac obliterated due to adhesion
between two layers of pericardium as well as
between parietal pericardium and
surrounding mediastinal structures, chest
wall & diaphragm.
 The heart thus contracts against all the
surrounding attached structures leading to
hypertrophy and dilation.

42.  3.Constrictive pericarditis:
 Marked thickening of parietal pericardium with
less involvement of visceral pericardium causing
constriction of great vessels entering and
leaving heart.
 Pericardial space obliterated by a dense fibrous
tissue, which is often calcified.
 Cardiac hypertrophy and dilation cannot occur
because of the dense enclosing scar and the
heart becomes smaller.
 Tuberculosis is the most common cause.

43.  Occasionally, may be due to pyogenic
infection and in some, the cause is unknown
but never rheumatic.
 The patients of pericarditis may develop
ascites and due to long standing ascites, liver
& spleen are coated with fibrin.
 Later, there is fibrosis of the liver (cardiac
cirrhosis).
 Pleura may be involved similarly.
 This polyserositis is known as Pick’s disease.

45. 45 of 48
CardiomyopathyCardiomyopathy

46.  Cardiomyopathies are diseases characterised
by cardiac dysfunction in which the main
abnormality lies in the myocardium.
 It is expressed as diastolic and /or systolic
ventricular dysfunction.
 Such abnormalities may directly affect one
or both ventricles in a diffuse or multifocal
fashion and in many patients produce
myocardial failure or arrythmias.

47.  Cardiomyopathies have been traditionally
divided into two main categories:
 (I) Primary -(idiopathic "heart muscle disease
of unknown causes“)
 (II) Secondary -("heart muscle diseases of
known cause or associated with disorders of
other systems).

48.  I Primary cardiomyopathy: -
 Dilated Cardiomyopathy : Familial ; Idiopathic.
 Hypertrophic cardiomyopathy : Obstructive ;
Nonobstructive ; Familial ; Nonfamilial.
 Restrictive cardiopathy : Endomyocardial
fibrosis ; Idiopathic.
 II Secondary cardiomyopathy:
 -Inflammatory ; Metabolic ;Toxic ; Infiltrative ;
Physical agents ; Neuromuscular disorders ;
Primary tumour of the myocardium ;
Hematologic ; Immunologically mediated
cardiomyopathy ; Miscellaneous.

49.  Based on pathophysiology there are three
major groups :
 1.Dilated ; 2.Hypertrophic ; 3.Restrictive .
 Dilated type - Hypertrophy & dilation of left
ventricle with normal left atrium. ;
 Hypertrophic type -Thick left ventricle with
small cavity but dilated left atrium. ;
 Restrictive type -Normal left ventricle but
dilated left atrium

51. Functional
Pattern LVEF *
Mechanisms of
Heart Failure Causes
Indirect
Myocardial
Dysfunction (Not
Cardiomyopathy)
Dilated <40% Impairment of
contractility
(systolic
dysfunction)
Idiopathic; alcohol;
peripartum; genetic;
myocarditis;
hemochromatosis;
chronic anemia;
doxorubicin
(Adriamycin);
sarcoidosis
Ischemic heart
disease; valvular
heart disease;
hypertensive
heart disease;
congenital heart
disease
Hypertrop
hic
50–80% Impairment of
compliance
(diastolic
dysfunction)
Genetic; Friedreich
ataxia; storage
diseases; infants of
diabetic mothers
Hypertensive
heart disease;
aortic stenosis
Restrictive 45–90% Impairment of
compliance
(diastolic
dysfunction)
Idiopathic;
amyloidosis;
radiation-induced
fibrosis
Pericardial
constriction

52. Cardiac Infections
Viruses
Chlamydia
Rickettsia
Bacteria
Fungi
Protozoa
Toxins
Alcohol
Cobalt
Catecholamines
Carbon monoxide
Lithium
Hydrocarbons
Arsenic
Cyclophosphamide
Doxorubicin (Adriamycin)
and daunorubicin
Metabolic
Hyperthroidism
Hypothyroidism
Hyperkalemia
Hypokalemia
Nutritional deficiency
(protein, thiamine, other
avitaminoses)
Hemochromatosis
Neuromuscular
Disease
Friedreich ataxia
Muscular dystrophy
Congenital atrophies
Storage Disorders and
Other Depositions
Hunter-Hurler syndrome
Glycogen storage disease
Fabry disease
Amyloidosis
Infiltrative
Leukemia
Carcinomatosis
Sarcoidosis
Radiation-induced
fibrosis
Immunologic
Myocarditis (several
forms)
Post-transplant rejection
Conditions asso.With heart
muscle diseases

53.  Characterized by slow, progressive dilation
of four chambers and contractile (systolic)
dysfunction;compensatory hypertrophy
 Age: May occur at any age (most common
20-60 years).
 Clinical presentation: Ineffective ventricular
systolic contraction causes congestive heart
failure, followed by embolic complications.

54.  Etiology:
 Cause is unknown.
 May be related to the effect of alcohol
toxicity, pregnancy or genetic defect.
 Heavy metal ingestion and some cytotoxic
drugs give an identitical picture.
 Idiopathic cases may be late sequela of viral
infection.

55.  Gross: Heart - Enlarged & flabby . Poor
contractile function and stasis can lead to the
formation of mural thrombi.
 -An increase in total heart weight due to
increase in left ventricular (LV) mass ;
 -Increase in LV cavity dimension ;
 -Normal or reduced LV wall thickness ;
 -LV endocardial thickening and mural
thrombi in atria or ventricles may or may not
be present.

56.  Microscopic features: The features include
permutations of the following features:
 1. Myocyte nuclear enlargement (polypoidy).
 2. Reduction myocyte width (attenuation)
 3. Loss of myofibrils in myocytes.
 4. Diffuse interstitial fibrosis.
 5. Increased numbers of intertitial
lymphocytes.

57. Path:
4 chamber dilatation
Hypertrophy
Interstitial Fibrosis
DCM

58.  Also termed as idiopathic hypertrophic
subaortic and hypertrophic obstructive
cardiomyopathy.
 The term hypertrophic cardiomyopathy is
used clinically to denote thick walled left
ventricle (LV) with a small cavity in which
systolic contraction is early and
discordinated, leading to outflow
obstruction.
 Diastolic relaxation is also impaired.

59.  Clinical presentation: Most patients remain
stationary and asymptomatic for years, but
some progressively worsen with major
complications.
 Outflow obstruction is seldom a major
problem.
 Complications : Atrial fibrillation with mural
thrombosis ; Embolization ; Infective
endocarditis ; Congestive heart failure ;
Sudden death.

60.  Gross: In hypertrophic cardiomyopathy, the
hypertrophy is not associated with ventricular
dilation.
 Disproportionate thickness of interventricular
septum and free wall of left ventricle cause
bulging of the interventricular septum into left
ventricle forming “banana-like” configuration of
left ventricle.
 NOTE:An increase in LV mass with a thick wall
and normal or reduced cavity can occur in 3
separate conditions.

61.  1. Appropriate LV hypertrophy - there is a
defined plausible cause i.e. aortic valve stenosis
or hypertension.
 The hypertrophy is microscopically normal.
 2. Inappropriate LV hypertrophy - there is either
no cause or a cause (mild hypertension)
insufficient in degree to responsible for the
degree of hypertrophy.
 The hypertrophy is microscopically normal.
Examples are renal hypertension, athletes hearts
and the hearts of infants of diabetic mother.

62.  3. Hypertrophic cardiomyopathy - In this
condition there is a histological abnormality -
Myocardial disarray with bundles running in
all directions and interstitial fibrosis.

63. 65% 35%
10%
www.kanter.com/hcm

64.  The condition is now recognised to be due to 5
different genes (heavy chain myosin ,TropininT ,
myosin binding protein C , tropomyosin and
preexcitation associated gene).
 The 4 known genes are concerned with myofibrillary
organisation within the myocyte and disorganisation
leads to misshapen myocytes runnning in whorls.
These genes are dominant negatives in which the
mutant protein interferes with the wild type protein
function.
 All the genes carry a risk of sudden unexpected death.
 .

65.  Cases with a large heavy thick walled LV
which may , or may not, be assymmetric are
relatively easy to recognise.
 About 30% of cases have a pathognomic
subaortic mitral impact lesion.
 A proportion of cases have normal or mild
increase in LV mass and macroscopically look
normal.
 Microscopy to identify these cases is needed.

66.  In this form of cardiomyopathy the abnormality
of function is in LV diastolic relaxation.
 The left atrial pressure rises in an effort to
achieve ventricular filling.
 This in turn leads to pulmonary hypertension.
The LV remains normal macroscopically, while
the right ventricle hypertrophies.
 The commonest cause is amyloid.
 Primary disease of the ventricle results in
impaired ventricular filling during diastole,
resulting in reduced cardiac outflow.
 This may be of the following types:

67.  Endomyocardial fibrosis:
 This is typically seen in children and young
adults in Africa.
 This is characterized by ventricular
subendocardial fibrosis, often with mural
thrombus formation.
 Reduced ventricular filling is due to reduced
ventricular chamber volume.

68.  Loeffler endocarditis:
 This is characterized by endo-myocardial fibrosis
with large mural thrombi and is found in
temperate zone.
 Eosinophilic infiltration is seen in multiple
organs including the heart.
 There may be associated peripheral
eosinophilia.
 Cationic proteins released from activated
eosinophils in the circulation damage the
endocardium.
 These lesions are rapidly fatal.

69.  Endocardial fibroelastosis:
 Characterized by focal-to-diffuse cartilage-like
fibroelastic thickening of the endocardium,
mostly of left ventricle.
 Mostly affected patients are younger than two
years old.
 Problems with Cardiomyopathy: In some
patients arrythmias and sudden death
predominate and contractile function is normal,
the subjects have normal exercise tolerance in
life.
 Several forms exist.

70.  - Arrhythmogenic right ventricular cardiomyopathy
: (arrhythmogenic right ventricular dysplasia).
 This is a familial disorder, most commonly associated
with right-sided heart failure and various rhythm
disturbances, particularly ventricular tachycardia and
sudden death.
 Morphologically, the dilated right ventricular wall is
severely thinned, with extensive fatty infiltration, loss
of myocytes and interstitial fibrosis.
 Death occurs due to congestive heart failure,
embolism of mural thrombi or fatal arrhthmias

71.  - In other subjects there is idiopathic fibrosis in
the left ventricle with a distribution and pattern
unlike ischaemic disease.
 It is not known whether this is acquired (? post
viral) or familial.
 - In the long QT syndrome (often referred to as
cardio-myopathy) now known to be due to
genetic defects in sodium and potassium
channels in the myocyte, sudden death is
common.
 The heart is normal structurally (macro and
micro).

72. Arrhythmogenic RightVentricular Cardiomyopathy
(Arrhythmogenic RightVentricular Dysplasia)
This is an uncommon dilated cardiomyopathy predominantly RIGHT ventricle.
So is NAXOSsyndrome.

73.  Acute non-specific myocarditis usually
produces rapid onset of fever, palpitations
and arrythmia with cardiac failure and a high
risk of sudden death.
 Pericarditis may or may not be present.
 Acute myocarditis is not an entity recognised
by macroscopic examination alone.

74.  Chiefly microbial
 COXACKIE A & B, CMV, HIV
 Trypanosoma cruzi (Chagas dis.), 80%
 Trichinosis
 Toxoplasmosis
 Lyme disease (5%)
 Diphtheria
 IMMUNE: Post-viral, rheumatic, SLE, drug
hypersensitivityalpha-methyl dopa, sulfas

75. LYMPHOCYTIC INFILTRATES are the USUAL pattern of ALL myocarditis, but eosinophils,
giant cells, and even trypanosomes can be seen occasionally

76.  Adriamycin
 Cyclophosphamide
 Catecholamines (Pheochromocytomas)
 Amyloid, systemic or primary cardiac
 Congo red stain: green birefringence with polarization
 Amyloid, aging
 Congo red stain: green birefringence with polarization
 Hemochromatosis (Prussian Blue)
 BOTH HYPER-, HYPO- -thyroidism

77.  Self-limiting
 Mild pleuritic chest pain
 Cardiac failure
 Endo-myocardial biopsy-lymphocytic infiltration
and myocyte necrosis

78.  Primary tumors of heart rare
 metastatic tumors to the heart occur in about
5% of patients dying of cancer
 most common primary tumors are
predominantly benign
 benign tumors account for 80-90% of
primary tumors

79.  myxomas,
 fibromas,
 lipomas,
 papillary fibroelastomas,
 rhabdomyomas,
 angiosarcomas and other sarcomas

80.  most common primary tumor of the heart in
adults
 90% are located in the atria,
 left-to-right ratio of approximately 4:1
(atrial myxomas).
 most are sporadic

81.  10% have a familial cardiac myxoma syndrome
(known as Carney syndrome)
 autosomal dominant transmission, multiple
cardiac and often extracardiac (e.g., skin)
myxomas, spotty pigmentation, and endocrine
overactivity
 gene PRKAR1 on chromosome 17 (encoding a
regulatory subunit of cAMP-dependent protein
kinase A, possibly a tumor suppressor gene) is
mutated in about half of known Carney complex
kindreds, while most of the other kindreds have
abnormalities in the locus 2p16

83.  most frequent primary tumor in infants and
children
 discovered in the first years of life because of
obstruction of a valvular orifice or cardiac
chamber.
 rhabdomyomas may be due to a defect in
apoptosis during developmental cardiac
remodeling

84.  Gross:small, gray-white myocardial masses up to
several centimeters in diameter
 located on either the left or the right side of the heart
and protruding into the ventricular chambers.
 M/E: mixed population of cells, the most
characteristic of which are large, rounded, or
polygonal cells containing numerous glycogen-laden
vacuoles separated by strands of cytoplasm running
from the plasma membrane to the more or less
centrally located nucleus, the so-called spider cells.
 These cells can be shown to have myofibrils.

85.  most frequent tumors involving the heart as
metastases are
 carcinomas of the lung and breast,
 melanomas,
 leukemias, and lymphomas

86.  retrograde lymphatic extension (most
carcinomas),
 hematogenous seeding (many tumors),
 direct contiguous extension (primary carcinoma
of the lung, breast, or esophagus), or
 by direct contiguous venous extension (tumors
of the kidney or liver).
 Clinical symptoms are associated with
pericardial spread, by either a pericardial
effusion that causes tamponade or by tumor
bulk that is sufficient to directly restrict cardiac
filling

87.  Pericarditis : Def,classification
 A)Eitiology:
 Infectitous-bacterial, viral ,fungal,parasitic
 Noninfectituos:immunological,systemic diseases
 B)Morphological-serous,fibrinous,purulent,hemorrhagic
 Cardiomyopathy:Types-Dilated/Hypertrophied/Restricted
 Myocarditis: causes
 Cardiac tumors:primary uncommon,
 Primary:Benign more common-
 Myxoma-adult
 Rhabdomyoma –infant,children
 Metastatic-Ca lung and breast, melanomas, leukemias,
and lymphomas