classifacation and basic intro. to CMP

1. Cardiomyopathies
Dr. Munaf Inamdar (MD Med.)
Associate Professor,
BHRC, Pune.

2. Definition
“A primary disorder of the heart muscle that causes
abnormal myocardial performance and is not the
result of disease or dysfunction of other cardiac
structures …
myocardial infarction,
systemic hypertension,
valvular stenosis or
regurgitation”

3. WHO Classification
 Unknown cause
(primary)
 Dilated
 Hypertrophic
 Restrictive
 Specific heart muscle
disease (secondary) due
to a known cause:
 Infective
 Metabolic
 Systemic disease
 Herediofamilial
 Sensitivity
 Toxic(alcohol)

4. Functional Classification
 Dilated (congestive, DCM, IDC)
 ventricular enlargement and syst dysfunction
 Hypertrophic (IHSS, HCM, HOCM)
 inappropriate myocardial hypertrophy
in the absence of HTN or aortic stenosis
 Restrictive (infiltrative)
 abnormal filling and diastolic function

5. We are going to cover….
 1. dilated cardiomyopathy (DCM)
 2. hypertrophic cardiomyopathy (HCM)
 3. restrictive cardiomyopathy (RCM)
And in addition…
 4. arrhythmogenic right ventricular dysplasia (ARVD)
 5. obliterative cardiomyopathy (OCM)

6. Diagramatic representation of different
cardiomyopathies

8. Idiopathic Dilated Cardiomyopathy
 a disease of unknown etiology that principally affects
the myocardium
 LV dilatation and systolic dysfunction
 pathology
 increased heart size and weight
 ventricular dilatation, normal wall thickness
 heart dysfunction out of portion to fibrosis

9. Most common cause of clinical
syndrome of Heart Failure

10. Incidence and Prognosis
 3-10 cases per 100,000
 20,000 new cases per year in the U.S.A.
 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

12. Symptoms.
 Symptoms of heart failure
 Due to pulmonary congestion (left HF)
dyspnea (rest, exertional, nocturnal), orthpnea
 Due to systemic congestion (right HF)
edema, nausea, abdominal pain, nocturia
 Due to low cardiac output
fatigue and weakness

13. Clinical Signs.
 Pulsus alternans
 Pulsus tardus
 Systolic blood pressure normal or less than normal.
 Raised JVP
 Prominent a and v waves tricuspid regurgitation
 b/l basal crepts
 Pedal edema
 Hepatomegaly
 Gallop rhythm
 Systolic murmurs—Mitral,tricuspid reg.

14. Findings on ECG
 Sinus tachycardia in presence of heart failure.
 Atrial and ventricular tachyarryhthmias
 Poor r wave progression
 Anterior q waves
 Intaventricular conduction defects –mostly LBBB
 Left atrial abnormality
 Hypertensive changes by voltage criteria not evident
 ST –T changes are seen.

15. Chest X Ray

16. Findings on 2D Echo.
 Dilated chambers
 Left atrium is usualy enlarged
 Left ventricle is enlarged.normal 3.8—5.0cm
 Mitral and tricuspid regurgitation on doppler flow.
 LV or Global hypokinesia with an LVEF of <45%
 Stress testing --tachyarryhthmias
 Dobutamine stress echo helpful in assessing the clinical
prognosis.

17. Treatment—
 ACE,
 B blockers in symptomatic,
 Diuretics- vol. overload,
 Spironolactone –advanced,
 Digoxin is drug of choice,
 Treatment of arrhythmias.
 Biventricular pacing—plus ICDs—IVCDS,
 Anticoagulation,
 Amiodarone,

18. Hypertrophic Cardiomyopathy

19. Anatomical subtypes of HCM

20. HCM: Incidence
 Most common cause of sudden death in young
athletes.
 Prevalence approx. 100 per 1 lakh population
 Characterized by in appropriate and elaborate LV
hypertrophy with misalignment of the myocardial
fibres.
 Hypertrophy may be generalized or confined largely to
interventricular septum.

21. Pathophysiology
 Heart failure may develop because stiff non-compliant
ventricles impede systolic filling.
 Septal hypertrophy may cause dynamic LV outflow
obstruction.
 Mitral regurgitation occur due to abnormal systolic
anterior mitral valve leaflet.

23. Clinical Features
 Symptoms…. S A D
 Syncope on effort/pre-syncope
 Angina on effort
 Dyspnea on effort
 Sudden death (SCD)
 (less frequent )…palpitations, PND, dizziness
 Signs
 Jerky pulse
 Palpable LV heave
 Double impulse at apex
 mid-systolic murmur –sign of LV outflow obstruction
 Pansystolic murmur

24. Risk factors for SCD
 Young age (<30 years)
 “Malignant” family history of sudden death
 Gene mutations prone to SCD (ex. Arg403Gln)
 Aborted sudden cardiac death
 Sustained VT or SVT
 Recurrent syncope in the young
 Nonsustained VT (Holter Monitoring)
 Brady arrhythmias (occult conduction disease)

25. Treatment
 B-blocker and rate-limiting calcium antagonists (eg.
verapamil)
 No pharmacological treatment is known to improve
prognosis
 Arrhythmia is common and responds well to amiodarone.
 Outflow tract obstruction can be improved by
 partial surgical resection (myectomy) or
 iatrogenic infarction of basal septum (septal ablation)
 Implantable cardiac defibrillation (ICD) for patient with
risk of sudden death.

26. Surgical intervention may better the prognosis

27. Restrictive Cardiomyopathy

28. Background
 Rare condition
 Ventricular filling is impaired because ventricles are
stiff.
 Lead to high atrial pressure with atrial hypertrophy,
dilatation and later atrial fibrillation.
 Amyloidosis is the most common cause.
 Other form:
 Idiopathic perimyocyte fibrosis
 Familial restrictive cardiomyopathy

29. Clinical Features
 Symptoms of right and left heart failure
 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)

30. Differential diagnosis
 Hallmark: abnormal diastolic function due to a rigid
ventricular wall with impaired ventricular filling.
 Bear some functional resemblance to constrictive
pericarditis.
 Importance lies in its differentiation from operable
constrictive pericarditis

31. Restrictive v/s Constrictive
History provide can important clues
 Constrictive pericarditis
 history of TB, trauma, pericarditis, collagen vascular
disorders
 Restrictive cardiomyopathy
 amyloidosis, hemochromatosis
 Mixed
 mediastinal radiation, cardiac surgery

32. Diagnosis and Treatment.
 Diagnosis is difficult
 Require complex Doppler echocardiography
 CT and MRI and endomyocardial biopsy may be done.
 No satisfactory medical therapy
 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
 Transplantation is the best treatment

33. Arrhythmogenic Right Ventricular Dysplasia
 In this condition, patches of the right ventricular myocardium
are replaced with fibrous and fatty tissue.
 Inherited as autosomal dominant trait
 Prevalence in India is 10 per 1 lakh population.
 Dominant clinical problems are
 ventricular arrhythmia
 Sudden death
 Right sided cardiac failure
 ECG shows inverted T waves in the right precordial leads.
 MRI useful diagnostic tool and used to screen 1st degree
relatives from having the same pathology.

34. Obliterative Cardiomyopathy
 It is also rare in India.
 Involves the endocardium of one or both ventricles and is
characterized by thrombosis and elaborate fibrosis with
gradual obliteration of the ventricular cavities.
 Mitral and tricuspid valves are regurgitant.
 Heart failure and pulmonary and systemic embolism are
prominent.
 Anticoagulation and antiplatelet therapy is advisable and
diuretics may help symptoms of HF.
 Surgery valve replacement with decortication of
endocardium may be helpful in certain cases.

35. A Quick Look

36. References:
 Harrison 18th Edtn.
 Braunwald heart disease 8th Edtn.
 Hurst Heart 12th Edtn.
 Jr. of the American Heart Association.
 British Medical Jr.
 Google.com for images.

37. ThankYou…