harrison based. AHA and ESC guidelines included

1. PRESENTER: DR. JITHIN GEORGE

2. heterogeneous group of diseases of the
myocardium associated with mechanical and/or
electrical dysfunction that usually (but not
invariably) exhibit inappropriate ventricular
hypertrophy or dilatation and are due to variety of
causes that frequently are genetic.
ischemic cardiomyopathy and nonischemic
cardiomyopathy

3.  classification of cardiomyopathies into a triad of
dilated, restrictive, and hypertrophic.
 four-way classification scheme of etiology as primary
(affecting primarily the heart) and secondary to other
systemic disease.
 The primary causes are then divided into genetic,
mixed genetic and acquired, and acquired

6.  the early symptoms often relate to exertional
intolerance with breathlessness or fatigue.
 peripheral edema may be absent despite severe fluid
retention, particularly in younger patients in whom
ascites and abdominal discomfort may dominate.

7. Most familial cardiomyopathies are
inherited in an autosomal dominant
pattern, with occasional autosomal
recessive and X-linked
Missense mutations with amino acid
substitutions are the most common in
cardiomyopathy
Mutations in sarcomeric genes, encoding
the thick and thin myofilament proteins,
are the best characterized esp in HCM

8. most commonly recognized
genetic causes of dilated
cardiomyopathy are structural
mutations of the giant protein
titin, encoded TTN.
Desmin mutations impair
the transmission of force and
signaling for both cardiac
and skeletal muscle and may
cause combined cardiac and
skeletal myopathy.

9. Sarcolemmal membrane protein defects are
associated with dilated cardiomyopathy.
The best known is dystrophin, encoded by the X
chromosome gene DMD
Defects in the sarcolemmal channel proteins
(channelopathies) are generally associated with
primary arrhythmias, but mutations in SCN5A,
implicated in dilated cardiomyopathy with
conduction disease.

11. Hypertrophic cardiomyopathy
 Defined as LV hypertrophy that develops in
absence of causative hemodynamic factors
such as HTN, aortic valve disease, systemic
infiltrative/storage disease.
 Also known as HOCM, asymmetric septal
hypertrophy and idiopathic hypertrophic sub-
aortic stenosis.
 Prevalence- 1in 500. Leading cause of sudden
cardiac death in young and is an important
cause of HF.
 Increased left ventricular wall thickness not
solely explained by abnormal loading
conditions
 ADULTS: LV wall thickness ≥15 mm in one or
more LV myocardial segments measured by any
imaging technique. CHILDREN: LV wall thickness
more than two standard deviations above the
predicted mean (z-score >2)

12.  Autosomal dominant inheritance. Clustering
within families is seen. Mutation in sarcomeric
genes present in 60% of HCM and most common
in asymmetric septal hypertrophy.
 80% have MYH7/MYBPC-3 mutations. Age
dependent and have incomplete penetrance.

14. Rarely presents at
birth. Develop later
part of life.
Screening should
begin in adolescence
and extend through
adulthood
Sarcomere mutation
causes increased
Ca2+ sensitivity,
maximal force
generation and
ATPase activity.
Abnormal
energetics and
impaired relaxation
due to mutation and
hypertrophy.
Characterised by
misalignment and
disarray of enlarged
myofibrils, fibrosis
and microvascular
changes.
Scar tissue are
substrate for
arrhythmias.

15. Increased thickness and
decreased luminal area
of intramural vessels in
hypertrophied area
cause microvascular
ischemia and angina.
Obstruction at rest-
30% of subjects and on
exertion other 30% of
subjects.
Systolic obstruction is
initiated by drag forces,
which push an
anteriorly displaced and
enlarged anterior mitral
leaflet into contact with
hypertrophied
ventricular septum.

18. Double apical impulse : a forceful
left atrial contraction against a
highly noncompliant left
ventricle.
Triple apical impulse : results
from a late systolic bulge
First heart sound is normal.
Second heart sound usually is
normally split, but in some
patients with severe outflow
gradients, it is paradoxically split.
An S3 gallop is common in
children. In adults:
decompensated congestive heart
failure. S4, frequently is heard
and results from atrial systole
against a highly noncompliant
left ventricle.[S4 >S3]
Jugular venous pulse reveals a
prominent- a wave : diminished
right ventricular compliance
secondary to massive
hypertrophy of the ventricular
septum

19.  Double carotid arterial pulse is common. The carotid pulse
rises quickly because of the increased velocity of blood
through the LV outflow tract and into the aorta. The
carotid pulse then declines in midsystole as the gradient
develops. This is followed by a secondary rise in carotid
pulsation during late systole.(Pulsus bisferens)
 Apical precordial impulse frequently is displaced laterally
and usually is abnormally forceful and enlarged.

20.  Systolic ejection murmur typically is a systolic
ejection crescendo-decrescendo murmur, which is
best heard between the apex and left sternal border
and radiates to the suprasternal notch but not to the
carotid arteries or neck.
 The murmur and the gradient across the LV outflow
tract diminish with any increase in preload
(squatting) or increase in afterload (handgrip). The
murmur and the gradient increase with any
decrease in preload (Valsalva manoeuvre, nitrate
administration, diuretic administration, standing) or
with any decrease in afterload (vasodilator
administration).

21. ECG
 Increased voltage in precordial leads
 Non specific ST-T changes. T wave inversions.
 In asymmetrical septal hypertrophy- deep narrow Q
waves in lateral and inferior leads that may mimic MI,
but Q wave duration <40ms.
 P mitrale in LA enlargement.
 Apical HCM- giant T in precordial leads
 Arrhythmias- SVT/AF.

26. LVOTO is defined as a peak instantaneous Doppler LV
outflow tract gradient of ≥30 mm Hg, but the
threshold for invasive treatment is usually considered
to be ≥50 mm Hg.

33. DILATED CARDIOMYOPATHY
An enlarged left ventricle with decreased systolic function
as measured by left ventricular ejection fraction.
Systolic failure is more marked than diastolic dysfunction.
Mitral regurgitation commonly develops as the valvular
apparatus is distorted and is usually substantial by the
time heart failure is severe.

39. MYOCARDITIS
 Infectious myocarditis has been reported with almost
all types of infective agents but is most commonly
associated with viruses and the protozoan
Trypanosoma cruzi.

40. Acute viral myocarditis often with symptoms and
signs of heart failure.
Some patients with chest pain suggestive of
pericarditis or acute myocardial infarction.
Occasionally, by atrial or ventricular tachyarrhythmias,
or by pulmonary or systemic emboli from intracardiac
thrombi.

41.  Chronic viral myocarditis is often invoked, but rarely
proven, as a diagnosis when no other cause of dilated
cardiomyopathy can be identified.
 However, some cases will later be recognized to have a
genetic basis, or ultimately found to have resulted
from excess alcohol consumption or illicit drugs.

42.  an ECG, an echocardiogram, and serum levels of
troponin and creatine phosphokinase fractions.
 Magnetic resonance imaging: increased tissue edema
and gadolinium particularly in the mid-wall (as
distinct from usual coronary artery territories)

44. The Dallas Criteria for
myocarditis on
endomyocardial biopsy
include lymphocytic
infiltrate with evidence
of myocyte necrosis
and are negative in 80–
90% of patients with
clinical myocarditis.
Immunohistochemistry
of myocardial biopsy :
to identify active
lymphocyte subtypes
and may also detect
upregulation of HLA
antigens and the
presence of
complement
components attributed
to inflammation.

45.  Possible subclinical acute myocarditis is diagnosed
when a patient has a typical viral syndrome but no
cardiac symptoms, with one or more of the following:
 • Elevated biomarkers of cardiac injury (troponin or CK-
MB)
 • ECG findings suggestive of acute injury
 • Reduced left ventricular ejection fraction or regional
wall motion
 • Abnormality on cardiac imaging, usually
echocardiography

46. 2. Probable acute myocarditis is diagnosed when the above criteria are
met and accompanied also by cardiac symptoms, such as shortness of
breath or chest pain, which can result from pericarditis or myocarditis.
When clinical findings of pericarditis (pleuritic chest pain, ECG
abnormalities, pericardial rub or effusion) are accompanie by elevated
troponin or CK-MB or abnormal cardiac wall motion, the terms
perimyocarditis or myopericarditis are sometimes used.
3. Definite myocarditis is diagnosed when there is histologic or
immunohistologic evidence of inflammation on endomyocardial
biopsy and does not require any other laboratory or
clinical criteria.

47.  First implicated was the picornavirus family of RNA
viruses, principally the enteroviruses, coxsackie virus,
echovirus, and poliovirus.
 Influenza, another RNA virus, is implicated with
varying frequency every winter and spring as epitopes
change.
 Of the DNA viruses, adenovirus, vaccinia (smallpox
vaccine), and the herpesviruses (varicella zoster,
cytomegalovirus, Epstein-Barr virus, and human
herpesvirus 6 [HHV6]) are well-recognized

48.  Human immunodeficiency virus (HIV) was associated
with an incidence of dilated cardiomyopathy.
 Hepatitis C has been repeatedly implicated in
cardiomyopathy

49. PERIPARTUM CARDIOMYOPATHY
Peripartum cardiomyopathy, a type of dilated
cardiomyopathy of unknown origin, occurs in
previously healthy women in the final month of
pregnancy and up to 5 months after delivery.
incidence is low—less than 0.1% of pregnancies
morbidity and mortality rates are high at 5% to
32%.

55. TOXIC CARDIOMYOPATHY
 ALCOHOL
 Toxicity due to alcohol and to its primary metabolite,
acetaldehyde
 Polymorphisms of the genes encoding alcohol
dehydrogenase and the angiotensin-converting
enzyme increase the likelihood of alcoholic
cardiomyopathy
 Atrial fibrillation occurs commonly both early in the
disease (“holiday heart”) and in advanced stages.
 Medical therapy includes neurohormonal antagonists
and diuretics as needed for fluid management.

56.  Cocaine, amphetamines, and related catecholaminergic
stimulants can produce chronic cardiomyopathy as
well as acute ischemia and tachyarrhythmias.
 Pathology reveals microinfarcts consistent with small
vessel ischemia, similar to those seen with
pheochromocytoma.

57. CHEMOTHERAPY
 Anthracycline: vacuolar degeneration and
myofibrillar loss. Generation of reactive oxygen species
involving heme compounds.
 Disruption of the large titin protein may contribute to
loss of sarcomere organization
 three different presentations of anthracycline-induced
cardiomyopathy:
 HF acute: singe dose
 Acute onset
 chronic

58. Doxorubicin cardiotoxicity leads to a relatively
nondilated ventricle, perhaps due to the accompanying
fibrosis.Thus, the stroke volume may be severely
reduced with an ejection fraction of 30–40%.
Therapy includes angiotensin-converting enzyme
inhibitors and β-adrenergic blocking agents used for
other causes of heart failure, with careful suppression
of “inappropriate” sinus tachycardia, and attention to
postural hypotension that can occur in these patients.

59. OTHER DRUGS
 Transtuzumab
 Cyclophosphamide and ifosfamide
 Interferon alpha

60. arrhythmogenic
cardiomyopathy.”
 Mutations in proteins of the desmosomal complex
compromise attachment of the myocytes replaced by
fat and fibrous tissue. These areas are highly
arrhythmogenic. more often noted in the right
ventricle (arrhythmogenic right ventricular
dysplasia),But can affect both ventricles

63. TAKOTSUBO CARDIOMYOPATHY
 Apical ballooning/stress induced cardiomyopathy typically
seen in older females after sudden intense emotional/physical
stress.
 Global ventricular dilation with basal contraction similar to
narrow necked jar used in japan for trapping octopus.
 C/F : pulmonary oedema, hypotension, chest pain, ECG
changes mimicking MI.
 LV dysfunction extends beyond a specific coronary artery
distribution and generally resolves within days to weeks may
result from intense sympathetic activation with heterogeneity of
myocardial autonomic innervation/diffuse coronary vasospasm
&/or catecholamine toxicity.
 Treatment: nitrates, intra aortic balloon pump for decreased
CO, α+β blockers, magnesium for arrhythmias.
 Anticoagulants are generally with-held due to occasional
ventricular rupture. Have good prognosis, recurs in up to 10%
patients.

66. Restrictive cardiomyopathy
 Least common cardiomyopathy
 Abnormal diastolic function, often with mildly
decreased contractility and EF (>30-50%)
 Both atria are massively enlarged, modest
ventricular dilation with end diastolic dimension
<6cm. End diastolic pressure increases with
preserved CO until late in disease.
 Subtle exercise intolerance is the first symptom,
later they develop right sided failure symptoms.
 Cardiac impulse is neither displaced nor
hyperdynamic, S4 +, AF is common, JVP rapid Y
descent, positive kussmaul’s sign.
 DD: constrictive pericardial disease.

68. 1. Amyloidosis- major cause of RCM
– Physical disruption and direct toxicity both affect cardiac function.
– Senile amyloidosis is associated with abnormal accumulation of normal
transthyretin/natriuretic peptide. 10% seen in >80 yrs and 50% in
>90 yrs age group without clinically apparent disease. Clinically
apparent disease is more common in males.
– 2D-echo show thick ventricular walls with refractile brightness in
septum
– ECG show low voltage complexes.
– MRI with gadolinium enhancement is useful in picking up the
lesions.
– Most reliably identified from heart biopsy.
– Treatment: loop diuretics, low dose digoxin, chronic anticoagulation.
– Prognosis worst among primary amyloidosis and best among senile
amyloidosis.
2. Fibrotic restrictive- progressive fibrosis. Seen post thoracic irradiation.
Present with possible diagnosis of constrictive pericarditis. Scleroderma
due small vessel spasm and ischemia have and small stiff heart.
Doxorubicin causes myocyte injury with increased fibrosis which
restricts remodelling and dilation.

69. 3. Endomyocardial disease- elevated filling pressures with atrial
enlargement, preserved ventricular contractility and with
normal/decreased ventricular volumes can result from
extensive fibrosis of endocardium without transmural
myocardial disease.
– Loeffler’s syndrome with eosinophil counts of >1500/mm3 for 6
months causing eosinophilic injury to endocardium cardiac
inflammation fibrosis with superimposed thrombosis dense
fibrotic layer can obliterate the ventricular apices and extend to
thicken and tether atrio-ventricular valve leaflets. Clinical
disease may present with heart failure, embolic events, atrial
arrhythmias.

70. – Treatment with glucocorticoids, chemotherapy,
anticoagulation, AF is difficult to suppress.
– Surgical resection can improve symptoms.

73. THANK YOU