The document discusses cardiomyopathies, defining them as diseases affecting the heart muscle, with classifications including primary and secondary types. It covers general presentations, management strategies, and specific types such as dilated, hypertrophic, and restrictive cardiomyopathy, emphasizing diagnosis and management approaches. Key symptoms, risk factors, and treatment options are also detailed, along with the importance of distinguishing between cardiomyomypathies and related conditions.

1. Cardiomyopathies
For Medical Students

2. Objectives
• Discuss definition, etiologies and classification of
CMP
• Review current medical management of patients with
cardiomyopathy

3. Definition
 A group of diseases that primarily affect the heart
muscle.
(not the result of disease or dysfunction of other cardiac
structures (valvular, hypertensive, coronary arterial, or
pericardial abnormalities).
 It is estimated that cardiomyopathy accounts for 5–
10% of the heart failure

4. Classification on an etiologic basis
• Primary type
- Heart muscle disease of unknown cause
• Secondary type
– Myocardial disease of known cause
– Associated with a disease involving other organ
system
– Specific cardiomyopathy by WHO

5. Peripartum heart disease(D)

8. WHO Classification
• Unknown cause
(primary)
• Dilated
• Hypertrophic
• Restrictive
• unclassified
• Specific heart muscle disease
(secondary)
• Infective
• Metabolic
• Systemic disease
• Heredofamilial
• Sensitivity
• Toxic

9. GENERAL PRESENTATION
• The early symptoms of cardiomyopathy
• exertional intolerance with breathlessness or fatigue.
• As filling pressures become elevated at rest, shortness of
breath may occur during routine activity or when lying down
at night.
• NB: Peripheral edema may be absent despite severe fluid
retention, particularly in younger patients in whom
abdominal discomfort from hepato-splanchnic congestion
and ascites may dominate.

10. GENERAL PRESENTATION
• May also present
• Atypical chest pain,
• palpitations
• syncope related to associated rhythm disorders, or
• with an embolism from an intracardiac thrombus.

11. Dilated Cardiomyopathy

12. IDCM - Definition
• a disease of unknown etiology that principally affects the
myocardium.
• pathology
• increased heart size and weight
• ventricular dilatation, normal wall thickness
• heart dysfunction out of portion to fibrosis

13. Peripartum cardiomyopathy (PPCM) develops during the last trimester
or within the first 6 months after pregnancy,

15. Idiopathic DCM
• Idiopathic DCM is a diagnosis of exclusion.
• Approximately two-thirds of DCMs are still labeled as idiopathic;
• however, a substantial proportion of these may reflect unrecognized
genetic disease.
• LV and/or right ventricular (RV) systolic pump function is impaired, leading to
progressive cardiac dilatation (remodeling).
• Symptoms of heart failure (HF) typically appear only after remodeling has been
ongoing for some time (months or even years).

16. Incidence and Prognosis
• 3-10 cases per 100,000
• Age: may occur at any age
• most commonly becomes apparent clinically in the 3rd or 4th decades.
• Blacks 2x more frequent than whites
• Men 3x more frequent than women
• Symptoms may be gradual in onset
• death from progressive pump failure
1-year 25%
2-year 35-40%
5-year 40-80%
• stabilization observed in 20-50% of patient
• complete recovery is rare

17. History and Physical Examination
• Symptoms of heart failure
• pulmonary congestion (left HF)
dyspnea (rest, exertional, nocturnal), orthpnea
• systemic congestion (right HF)
edema, nausea, abdominal pain, nocturia
• low cardiac output
fatigue and weakness
• Hypotension, narrow pulse pressure, tachycardia,
tachypnea, JVD, S3,S4, MR/TR

18. Cardiac Imaging
• Chest radiogram:
• enlargement of the cardiac silhouette
• Pulmonary vascular redistribution and interstitial or, in advanced cases,
alveolar edema.
• Electrocardiogram:
• sinus tachycardia or atrial fibrillation, ventricular arrhythmias, left atrial
abnormality, low voltage, diffuse nonspecific ST-T-wave abnormalities, and
sometimes intraventricular and/or AV conduction defects.
• Echocardiography, computed tomographic imaging (CTI), and
cardiac magnetic resonance imaging (CMRI)
• Radionuclide ventriculography
• Cardiac catheterization and coronary angiography
• age >40, ischemic history, high risk profile, abnormal ECG

19. Management of DCM
• Supportive
• salt restriction of a 2-g Na+ (5g NaCl) diet
• Oxygen (if indicated)
• Activity (might be limited based on functional status, elevate head of bed)
• Pharmacologic treatment
• Decongest with Diuretics
• Based on Ejection Fraction and comorbidities
• MRA
• ARNI/ACEI/ARB
• ß-blocking agents
• SGLT2I
• Treat complication like arrhythmia
• Treat precipitants

20. Hypertrophic Cardiomyopathy

21. Hypertrophic Cardiomyopathy
• A disorder caused by mutations in the genes that code for
various proteins within the sarcomere.
• uncommon with occurrence of 0.02 to 0.2%
• a hypertrophied and non-dilated left ventricle in the absence of
another disease
• small LV cavity, asymmetrical septal hypertrophy (ASH), systolic
anterior motion of the mitral valve leaflet (SAM)

22. Pathophysiology
• Systole
• dynamic outflow tract gradient
• Diastole
• impaired diastolic filling,  filling pressure
• Myocardial ischemia
•  muscle mass, filling pressure, O2 demand
•  vasodilator reserve, capillary density
• abnormal intramural coronary arteries
• systolic compression of arteries

23. Clinical Manifestation
• About half of all patients with HCM have a positive
family history compatible with autosomal dominant
transmission.
• Asymptomatic, echocardiographic finding
• Symptomatic
• dyspnea in 90%
• angina pectoris in 75%
• fatigue, pre-syncope, syncope
 risk of SCD in children and adolescents
• palpitation, PND, CHF, dizziness less frequent

24. Physical Examination
• Most patients demonstrate a double or triple apical precordial
impulse and a fourth heart sound.
• a rapidly rising arterial pulse: Those with intraventricular pressure
gradients.
• systolic murmur:
• the hallmark of obstructive HCM, which is typically harsh, diamond-
shaped, & usually begins well after 1st
heart sound.

25. Laboratory Evaluation
• ECG: LV hypertrophy and widespread deep, broad Q
waves, T wave inversions.
• Chest X-ray: may be normal, although a mild to moderate
increase in the cardiac silhouette is common.
• Echocardiogram: The mainstay of the diagnosis of HCM
• LV hypertrophy, often with the septum 1.3 times the
thickness of the posterior LV free wall.
• CMRI is superior to echocardiography in providing
accurate measurements of regional hypertrophy and in
identifying sites of regional fibrosis.

26. Natural History
• annual mortality 3% in referral centers probably
closer to 1% for all patients
• risk of SCD higher in children may be as high as 6%
per year majority have progressive hypertrophy
• clinical deterioration usually is slow
• progression to DCM occurs in 10-15%

27. Management
• Management focuses on treatment of symptoms and
prevention of sudden death and stroke.
• Left ventricular outflow tract obstruction can be
controlled medically in the majority of patients.
• β-Adrenergic blocking agents and L-type calcium
channel blockers (e.g., verapamil) are first-line agents
that reduce the severity of obstruction by slowing
heart rate, enhancing diastolic filling, and decreasing
contractility.

30. Recommendations for competitive
Activity
• Avoid most competitive sports (whether or not symptoms and/or outflow
gradient are present)
• Low-risk older patients (>30 yrs) may participate in athletic activity if all of the
following are absent
• ventricular tachycardia on Holter monitoring
• family history of sudden death due to HCM
• history of syncope or episode of impaired consciousness
• severe hemdynamic abnormalities, gradient 50 mmHg
• exercise induced hypotension
• moderate or sever mitral regurgitation
• enlarged left atrium (50 mm)
• paroxysmal atrial fibrillation
• abnormal myocardial perfusion

31. Restrictive Cardiomyopathy

32. Restrictive Cardiomyopathies
• Hallmark: abnormal diastolic function
• rigid ventricular wall with impaired ventricular filling
• bear some functional resemblance to constrictive
pericarditis
• importance lies in its differentiation from operable
constrictive pericarditis

33. Exclusion “Guidelines”
• LV end-diastolic dimensions  7 cm
• Myocardial wall thickness  1.7 cm
• LV end-diastolic volume  150 mL/m2
• LV ejection fraction < 20%

34. Clinical Manifestations
• The restrictive diseases often present with relatively
more right sided.
• Symptoms, such as edema, abdominal discomfort, and
ascites, although filling pressures are elevated in both
ventricles.
• The cardiac impulse is less displaced than in DCM and
less dynamic than HCM.
• S4 is more common than S3 in sinus rhythm, but atrial
fibrillation is common.

35. Clinical Manifestations
• Jugular venous pressures often show rapid Y descents
and may increase during inspiration (positive
Kussmaul’s sign).
• Jugular Venous Pulse
• prominent x and y descents
• Echo-Doppler
• abnormal mitral inflow pattern
• prominent E wave (rapid diastolic filling)
• reduced deceleration time ( LA pressure)

36. Causes of Restrictive
Cardiomyopathy
• Most restrictive cardiomyopathies are due to
infiltration of abnormal substances between
myocytes, storage of abnormal metabolic products
within myocytes, or fibrotic injury
• Amyloidosis is the most common cause of restrictive
cardiomyopathy.

38. Restriction vs Constriction
History provide important clues
• Constrictive pericarditis
• history of TB, trauma, pericarditis, collagen vascular disorders
• Restrictive cardiomyopathy
• amyloidosis, hemochromatosis
• Mixed
• mediastinal radiation, cardiac surgery

39. Laboratory Examinations
• ECG: low-voltage, nonspecific ST-T-wave abnormalities and
various arrhythmias.
• Chest x-ray: Pericardial calcification which occurs in
constrictive pericarditis, is absent.
• Echocardiography, CTI, and CMRI typically reveal
symmetrically thickened LV walls and normal or slightly reduced
ventricular volumes and systolic function; the atria are usually
dilated, sometimes massively.

40. Laboratory Examinations…
• Endomyocardial biopsy: reliable for the Dx of all amyloid due
to the characteristic birefringence pattern of Congo red staining
of the amyloid fibrils
• Doppler echocardiography typically shows diastolic
dysfunction.
• Cardiac catheterization shows a reduced CO, elevation of the RV
and LV end-diastolic pressures, and a dip-and-plateau
configuration (resembling constrictive pericarditis)

41. Treatment
• Medical therapy is not satisfactory
• Anticoagulation is recommended to reduce the risk of
embolization from the heart.
• Drug therapy must be used with caution
• diuretics for extremely high filling pressures
• vasodilators may decrease filling pressure
• ? Calcium channel blockers to improve diastolic compliance
• digitalis and other inotropic agents are not indicated