CARDIOMYOPATHY

April Grace A. Chiongbian

 Group of diseases that primarily affect the heart muscle. Not a result of congenital, acquired, valvular, hypertensive, coronary arterial or pericardial abnormalities

Three Morphologic Types

Dilated Cardiomyopathy Restrictive Cardiomyopathy Hypertrophic Cardiomyopathy

EPIDEMILOGY
Endomyocardial Fibrosis
Children and young adults Tropical and subtropical Africa Frequent cause of CHF in Africa

Dilated Cardiomyopathy in Africa

Infection with Toxoplasma gondii and Coxsackievirus B (Nigeria) African Trypanosomiasis (Cameroon) of patient hospitalized with CHF

 Peripartum DCM
More common in Africa than in North Aerica and Europe
Low socio-economic Status High Parity Excessive Salt intake Selenium deficiency Prolonged lactation

 Cardiomyopathy in Asia
Thiamine Deficiency
Wet beri-beri heart disease

Selenium Deficiency
Keshans Disease\

Commonly presents in 3rd to 4th decade of life

DILATED CARDIOMYOPATHY

 Ventricular chamber enlargement Systolic pump function is impaired leading to progressive cardiac dilatation

Etiology
Familial End result of myocardial damage due to known or unknown Infectious, metabolic or toxic agents Late consequence of acute viral myocarditis Reversible form may be found with alcohol abuse, pregnancy, thyroid disease, cocaine use and chronic uncontrolled tachycardia

Alcoholic Cardiomyopathy Peripartum Cardiomyopathy Neuromuscular Diseases Drugs Arrhythmogenic Right Ventricular Cardiomyopathy/ Dysplasia (ARVC/D)

Clinical Features
Symptoms develop gradually LV dilatation occurs for months or years before becoming symptomatic Vague chest pain Syncope due to arrythmias and systemic embolism

Physical Examination
Narrow pulse pressue Jugular venous pressure is elevated Third and fourth heart sounds Mitral or tricuspid regurgitation

Laboratory Examination
Chest Roegenogram enlargement of cardiac sillhouette ECG left bundle branch block and ST-T wave abnormality CTI and CMRI LV dilatation Cardiac Catheterization Coronary angiography dilated, diffusely hypokinetic left ventricle

ALCOHOLIC CARDIOMYOPATHY
>90g/d of alcohol Risk of developing cardiomyopathy is partially determined genetically Polymorphism of the gene encoding alcohol dehydrogenase as well as the DD form of ACE gene Poor prognosis (<1/4 survive 3 years) Management: Abstention

 Holiday Heart Syndrome

Second presentation of alcoholic cardiotoxicity Recurrent supraventricular or ventricular tachyarrhythmias Appears after a drinking binge Atrial fibrillation Atrial flutter Frequent ventricular premature depolarizations

PERIPARTUM CARDIOMYOPATHY
Last trimester of pregnancy or within 6 months of delivery Cause: Inflammatory myocarditis, immune activation and gestational hypertension Multiparous, African ancestry, >30 years old Prognosis:
Poor: LV remains enlarged and/ or LV ejection fraction remains depressed after 6 months

NEUROMUSCULAR DISEASE
In Duchennes progressive muscular dystrophy, mutations in genes that encode dystrophin lead to mycocyte death Tall R waves in right precordial leads with an R/S ration >1.0, often associated with deep Q waves in limb and lateral precordial leads Supraventricular and ventricular arrhythmias

 Myocardial Dystrophy
Disorders of impulse formationn and AV conduction Syncope ad sudden death are major haards Insertion of an ICD and/or permanent pacemaker

DRUGS
Anthracycline derivatives
Systolic dysfunction and ventricular arrhythmias Cardiotoxicity 3 months after last dose Late contractile dysfunction Prevention: document preclinical deterioration of LV function Modification of dose and dose schedule Take along with cardioprotective agents (iron-chelator dexrazoxone) ACE inhibitors

Trastuzumab Cyclophosphamide Imatinib mesylate Cocaine abuse

Cardiac complications: SCD, Myocarditis, DCM and acute MI Nitrates, calcium channel blockers, antiplatelet agents and benzodiazepines

Arrhythmogenic Right Ventricular Cardiomyopathy/ Dysplasia (ARVC/D)

Familial Progressive fibrofatty replacement of the right ventricle and LV myocardium Autosomal dominant Mutations in desmosomes Plakophilin-2 most common

RV failure with Jugular venous distention Hepatomegaly Edema Manifest during 2nd decade
Ventricular tachyarrhythmias RV failure

ECG
QRS prolongation

CT-Scan/ MRI
RV dilatation, RV aneurysm ad Fatty replacement MANAGEMENT: Restriction from Competitive sports Antiarrhythmic therapy with beta blockers or amiodarone

TAKO-TSUBO (stress) CARDIOMYOPATHY

Apical Ballooning syndrome Abrupt onset of severe chest discomfort Preceded by very stressful emotional or physical event Women >50 y/o ST segment elevations; Deep T wave inversions Severe akinesia of LV Troponins mildly elevated
Reversible within 3-7 days Adrenergic Surge involved mechanism

LEFT VENTRICULAR NONCOMPACTION

Uncommon congenital Present at any age (CHF, thromboembolism, Ventricular arrhythmias) Arrest of normal embryogeneis Echocariography
Multple deep trabeculations into the myocardium

TREATMENT
Standard therapy for Heart Failure Cardiac Resynchronization Therapy Insertion of Implantalbe Cardioverter Defibrillator Cardiac Transplantation

HYPERTROPHIC CARDIOMYOPATHY

 LV hypertrophy Two Features: Asymmetric LV hypertrophy Dynamic LV outflow tract pressure gradient

Diastolic Dysfunction ubiquitous pathophysiologic abnormality Distinctive pattern of hypertrophy

CLINICAL FEATURES
First Clinical Manifestation: Sudden Cardiac Death Dyspnea most common complaint Syncope Angina pectoris Fatigue

PHYSICAL EXAMINATION
Double or triple apical precordial impulse Fourth heart sound Systolic Murmur
Hallmark of obstructive HCM Typically harsh, diamond-shaped and begins well after the first heart sound.

HEMODYNAMICS
Pressure gradient is dynamic Obstruction is due to narrowing of LV outflow tract by systolic anterior movement of the mitral valve against the hypertrophied septum Three basic mechanisms:
Increased LV contractility Decreased ventricular preload Decreased aortic impedance and pressure

LABORATORY EXAMINATIONS
ECG: LV hypertrophy and widespread, deep broad Q waves. Echocardiogram
Mainstay of diagnosis LV hypertrophy, septum <1.3 times the thickness of the posterior LV free wall. Septum demonstrates :ground-glass: appearance

PROGNOSIS
SCD is major cause of mortality Atrial Fibrillation common late in the disease Infective Endocarditis <10% of patients

INHERITED METABOLIC CM with LV HYPERTROPHY

Cardiac Danon Disease Glycogen Storage Cardiomyopathy Fabry Disease Friedreichs Ataxia

TREATMENT
B- adrenergic blockers Amiodarone Nindihydropyridine calcium channel blockers (verapmil and diltiazem) Surgical Myotomomy/ Myectomy Insertion of ICD

RESTRICTIVE CARDIOMYOPATHY

 Abnormal diastolic function Ventricular walls are excessively rigid and impede ventricular filling Partial obliteration of the ventricular cavity by fibrous tissue and thrombus contributes to the abnormally increased resistance

CLINICAL FEATURES
Exercise intolerance Dyspnea Dependent edema Ascites Enlarged tender and often pulsatile liver Elevated jugular venous pressure and does not fall normally with inspiration (Kussmauls sign) 3rd and 4th heart sounds Apex impulse easily palpable Mitral regurgitation is common

LABORATORY EXAMINATION
ECG: Low voltage, nonspecific ST-T wave abnormalities
and various arrhythmias

Echocardiography, CTI and CMRI: symmetrically

thickened LV walls and normal or slightly reduced ventricular volumes and systolic function; Atria usually dilated

Doppler: Diastolic Dysfunction Cardiac Catherterization: reduced cardiac output,

elevation of LV and RV end-diastolic pressures, and a dipand-plateau configuration

ETIOLOGIES
Eosinophilic Endomyocardial Disease Cardiac Amyloidosis

EOSINOPHILIC ENDOMYOCARDIAL DISEASE

Loefflers endocarditis or Fibroplastic Endocarditis Occurs in temperate climates Endocardium of either or both ventriles is thickened Mitral regurgitation present on Doppler Large mural thrombi may develop in either ventricle Hepatosplenomegaly and localized infiltration of other organs are present Management: Diuretics, afterload-reducing agnets and anticoagulation

CARDIAC AMYLOIDOSIS
Four clinical presentations:
Diastolic dysfunction Systolic dysfunction Arrhythmias and conduction disturbances Orthostatic hypotension

2d-Echo: show thickened myocardial wall with a diffuse, hyperrefractile speckled appearance Management: Chemotherapy, Heart transplantation

Other RCM
Iron-overload cardiomyopathy (hemochromatosis) Myocardial Sarcoidosis Carcinoid Syndrome

TREATMENT
Salt restriction Diuretics Digitalis impaired systolic function Anticoagulation eosinophilic endomyocarditis

OT Management
Energy Conservation Techniques Relaxation Techniques Patient Education and Supportive Therapy