Cardiomyopathy

CARDIOMYOPATHY Bernardo D. Morantte Jr. M.D. Dept. of Medicine College of Medicine Pamantasan Ng Lungsod Ng Maynila

Cardiomyopathy • Definition: It is an abnormality in the diastolic and /or systolic functions and properties of the heart, primarily affecting the cardiac musculature, in the absence of primary valvular pathology, congenital anomalies, atherosclerotic coronary artery disease and hypertension.

Cardiomyopathy • General characteristics: • Its main presentation is congestive heart failure although it may occur late in the disease process. • When the cause is unknown, it is referred to as primary type. When the cause is known it is called secondary type.

Classification of Cardiomyopathy (based on hemodynamics and pathology) •

Dilated (congestive ) both ventricles are enlarged although initially it may

affect only the left. 4. Hypertrophic hypertrophy of both ventricles although it may predominantly affect the left. Assymetric septal hypertrophy (ASH) is present a. Obstructive ( HOCM or IHSS) b. Non-obstructive 3. Restrictive or infiltrative Infiltration of the myocardium with abnormal substances resulting in hypertrophy of both ventricles.

Hemodynamic differences TYPES Dilated Hypertrophic Restrictive Systolic function: abnormal normal normal ( Ejection fraction)

Diastolic function: abnormal abnormal abnormal Outflow obstruction: none

SAM*

none

*SAM – systolic anterior movement of the mitral valve (dynamic obstruction) as seen in the echo.

HOCM AORTA AV

SAM

LVOT obstruction ASH RV

LA MV

LV

Symptoms Dilated Dyspnea / easifatigability Orthopnea/PND

Hypertrophic late

Restrictive also present

Late or none

present

None Palpitation

+Angina Also present

Atypical chest pain Also present

Syncope due to ventricular arrhythmias Abdominal pain due to hepatomegaly

Syncope due to Syncope due to LVOT AV block obstruction none Also present

PE Findings Dilated

Hypertrophic

Restrictive

Low BP

Normal BP

Normal / low BP

JVP large V waves Displaced apical impulse + MR, TR

Normal

JVP prominent A wave normal

+Apical S3

Double apical thrust SEM at LSB, +MR + apical S4

Signs of CHF

CHF is late

+ MR, TR Right sided/ left sided S3, S4 + + CHF _maybe right sided only

Diagnostics Chest x-ray EKG Dilated

Hypertrophic

•Marked cardiomegaly • pleural effusion

Normal heart size

Restrictive •Enlarged

heart /normal •Hilar adenopathy

Echo

• Bi-ventricular dilatation •Low amplitude infarct pattern of motion of MV • reduce EF •Deep Q waves • SAM of MV •LVH, giant T • ASH wave inversion • Normal EF

•GIVCD • Pseudo-

•Low QRS voltage • ST and T wave changes

• Hypertrophy / obliteration of the ventricle • Normal EF / slight reduced

Diagnostics Doppler

Cardiac cath

Endocardial BX

Dilated

• + MR, TR • Abnormal diastolic flow in the MV

• Elevated LVEDP

Hypertrophic

• + MR • Pressure gradient at LVOT

• Dynamic Not helpful obstruction at LVOT • Double chamber appearance of LV in angiogram • Square root Evidence for sign in the myocardial ventricular infiltration pressure tracing

Restrictive • MR, TR • abnormal diastolic flow in the MV

Not helpful

Causes of Dilated Cardiomyopathy 1. 2. 3.

7. 8. 9. 10. 11. 12. 13. 14.

Post- inflammatory / post-infectious Cardiotoxic substances _ alcohol, lead, hydrocarbons Drugs: a. Psychiatric meds_ Phenothiazines, Lithium, Tri-cyclic antidepressants b. Anti-cancer drugs_ adriamycin, cyclophospamide, dauronobicin Nutritional deficiencies Metabolic abnormalities Endocrine disorders _ diabetes, hypothyroidism, acromegaly Heat stroke / hypothermia Radiation Post partum cardiomyopathy Connective tissue disorders, LE, RA etc.

Causes of Hypertrophic* cardiomyopathy • Familial • Idiopathic • Freiderich’s ataxia

• * Must be differentiated from hypertensive heart disease.

Causes of Restrictive cardiomyopathy 1. 2. 3. 4. 5. 6. 7. 8. 9. 10.

Amyloidosis Sarcoidosis Hemosiderosis Carcinoid syndrome Hypereosinophic syndrome (Loeffler’s endocarditis) Glycogen storage diseases Endomyocardial fibroelastosis Fabry’s disease Idiopathic Post radiation

Therapy for dilated cardiomyopathy 1. 2. 3. 4.

Withdrawal of toxic substances ex. Alcohol Treatment of specific metabolic abnormalities Treatment of nutritional deficiencies Rx: for CHF Digitalis Loop and K sparing diuretics 5. After load reducing agents with ACE inhibitors 6. Beta blockers 7. Anti- arrhythmic medications for A-fib, SVT, VT

Therapy for Hypertrophic Cardiomyopathy • Betablockers such as Inderal • Myomectomy / myotomy • Family screening

Therapy for restrictive cardiomyopathy Specific therapy depends on etiology • Hemochromatosis: Stop Fe supplements, reduce blood transfusions, chelation Rx • Steroid Rx for Sarcoidosis, Loeffler’s endocarditis • Betablockers • Rx for CHF* caution on digitalis in amyloidosis • Surgical relief

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