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Bernardo D. Morantte Jr. M.D. Dept. of Medicine College of Medicine Pamantasan Ng Lungsod Ng Maynila
• 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.
• 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.
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.
AORTA AV SAM LA MV
LVOT obstruction ASH RV
Dilated Dyspnea / easifatigability Orthopnea/PND None Palpitation Syncope due to ventricular arrhythmias Abdominal pain due to hepatomegaly Hypertrophic late Late or none +Angina Also present Restrictive also present present Atypical chest pain Also present
Syncope due to Syncope due to LVOT AV block obstruction none Also present
Dilated Low BP JVP large V waves Displaced apical impulse + MR, TR +Apical S3 Signs of CHF Hypertrophic Normal BP Normal Double apical thrust SEM at LSB, +MR + apical S4 CHF is late Restrictive Normal / low BP JVP prominent A wave normal + MR, TR Right sided/ left sided S3, S4 + + CHF _maybe right sided only
Diagnostics Chest x-ray EKG
Dilated •Marked cardiomegaly • pleural effusion •GIVCD • Pseudo-
Normal heart size
• 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 •Low QRS voltage • ST and T wave changes
heart /normal •Hilar adenopathy
• Hypertrophy /
obliteration of the ventricle • Normal EF / slight reduced
Doppler Cardiac cath
• Elevated LVEDP
• + MR, TR • Abnormal diastolic flow in the MV • + MR • Pressure gradient at LVOT
Restrictive • MR, TR
• abnormal diastolic flow in the MV
• 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
Causes of Dilated Cardiomyopathy
1. 2. 3. 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.
7. 8. 9. 10. 11. 12. 13. 14.
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