CARDİOMYOPATHİESCARDİOMYOPATHİES

Definition:Definition:

Cardiac dysfunction caused by myocardial disease.Cardiac dysfunction caused by myocardial disease.

Five Categories:Five Categories:1-1- Dilated Cardiomyopathy ( Dilated Cardiomyopathy ( geneticgenetic ++).).2- 2- Hypertrophic Cardiomyopathy ( Hypertrophic Cardiomyopathy ( genetic +genetic +).).3- 3- Restrictive Cardiomyopathy.Restrictive Cardiomyopathy.4- 4- Arrythmic Cardiomyopathy (Arrythmic Cardiomyopathy (geneticgenetic ++).).5-5- Unclassified Cardiomyopathy .Disease has not fature Unclassified Cardiomyopathy .Disease has not fature

1- 4 (Fibroelastosis and Mitochondrial disease)1- 4 (Fibroelastosis and Mitochondrial disease)

- Some of the Infiltrative and systemic diseases cause - Some of the Infiltrative and systemic diseases cause Spesific heart muscle disease called: Spesific heart muscle disease called: “Secondary “Secondary Cardiomyopathy” or “Specific Cardiomyopathy”.Cardiomyopathy” or “Specific Cardiomyopathy”.

Secondary Myocardial DiseasesSecondary Myocardial Diseases

Definition:Definition:Myocardial disease of known origin. Myocardial disease of known origin.

((Secondary CardiomyopatySecondary Cardiomyopaty))

7 Subgroups:7 Subgroups:1-1- Ischemic Cardiomyopathy Ischemic Cardiomyopathy2-2- Hypertensive Cardiomyopathy Hypertensive Cardiomyopathy3-3- Valvular Cardiomyopathy Valvular Cardiomyopathy4-4- Alcoholic Cardiomyopathy Alcoholic Cardiomyopathy5-5- Metabolic Cardiomyopathy Metabolic Cardiomyopathy6-6- Muscular Distrophy Cardiomyopathy Muscular Distrophy Cardiomyopathy7-7- Peripartum Cardiomyopathy Peripartum Cardiomyopathy

HYPERTROPHİC CARDİOMYOPATHY (HCM)HYPERTROPHİC CARDİOMYOPATHY (HCM) Definition:Definition: Massive ventricular hypertrophy with no Massive ventricular hypertrophy with no

absolute cause.absolute cause.Not all of these patients show Not all of these patients show IHSSIHSS (Idiopatic (Idiopatic Hypertrophic Subaortic Stenosis) or Hypertrophic Subaortic Stenosis) or HOCMP HOCMP (Hypertrophic Obstructive Cardiomyopathy) features. (Hypertrophic Obstructive Cardiomyopathy) features. There is no pressure gradient at left ventricle There is no pressure gradient at left ventricle outflow(Loutflow(LVOTVOT) tract in 1/3 of patient. (Rest or ) tract in 1/3 of patient. (Rest or provocated gradient).provocated gradient).

HCMP prevalance:HCMP prevalance: 1/500. HCMP is the most prevalant 1/500. HCMP is the most prevalant genetic cardiovascular disease. genetic cardiovascular disease. Nearly %60 inherited.Nearly %60 inherited. Relation with mutation of beta-Relation with mutation of beta-myosin heavy chain and also other sarcomeric proteins myosin heavy chain and also other sarcomeric proteins has been shown. (Troponin- T, -I, -C, Myosin Related has been shown. (Troponin- T, -I, -C, Myosin Related Protein- C etc.). Protein- C etc.). HCM ıs the most frequent cause of sudden cardiac HCM ıs the most frequent cause of sudden cardiac death among young people including athletes.death among young people including athletes.

Structure of Human Sarcomere:Structure of Human Sarcomere: Contraction is carried Contraction is carried out by the interaction of actin and myosin. out by the interaction of actin and myosin.

The process begins with the ligation of calcium to troponin The process begins with the ligation of calcium to troponin complex (I, C, T) and alpha-tropomyosin. Then, actin binds to complex (I, C, T) and alpha-tropomyosin. Then, actin binds to myosin and activates ATPase of the spheric myosin, so that myosin and activates ATPase of the spheric myosin, so that contractile function is carried out. Cardiac myosin binded contractile function is carried out. Cardiac myosin binded protein C binds to myosin and starts contraction.protein C binds to myosin and starts contraction.

HYPERTROPHIC CARDIOMYOPATHY: HYPERTROPHIC CARDIOMYOPATHY: Cellular Cellular changes of myocytes. Cell disarrangement ise seen changes of myocytes. Cell disarrangement ise seen on microscope. on microscope. Left:Left: Organized and parallel cell Organized and parallel cell arrangement in normal myocardium. arrangement in normal myocardium. Right:Right: Disarrangement affects impuls conduction and Disarrangement affects impuls conduction and promotes ventricular arrythmias. promotes ventricular arrythmias.

Common HCM types:Common HCM types: (A)-(A)- LV free wall, diffusely hypertrophied İVS. LV free wall, diffusely hypertrophied İVS. (B)-(B)- Asymmetric septal hypertrophy Asymmetric septal hypertrophy (C)-(C)- MassiveConcentric LV MassiveConcentric LV

hpertrophy ( involve walls, papillary muscles).hpertrophy ( involve walls, papillary muscles).

(A)-

(B)- -(C)

HCMP: PatophysiologyHCMP: Patophysiology

Most patients have septum hypertrophy with non-dilated LV and/or RV cavity. Most patients have septum hypertrophy with non-dilated LV and/or RV cavity. Hypertrophy of the septum may be diffuse or only the upper, mid or apical Hypertrophy of the septum may be diffuse or only the upper, mid or apical

hypertrophy of the septum may take place. hypertrophy of the septum may take place. Hypertrophy is extednded to the LV free wallHypertrophy is extednded to the LV free wall in most of the patients. in most of the patients. Diastolic filling is impairedDiastolic filling is impaired because of the incomplete relaxation and because of the incomplete relaxation and compliance of the LV. compliance of the LV. Hypertrophic LV empties most of its content in the first half of systole. Hypertrophic LV empties most of its content in the first half of systole. ((Hyperdynamic systolic function”).Hyperdynamic systolic function”). Mitral anterior leaflet is displaced towards septum during mid systole and this Mitral anterior leaflet is displaced towards septum during mid systole and this causes causes obstruction the the LV outflow tractobstruction the the LV outflow tract during this period. during this period. At the obstructive phase of the disease At the obstructive phase of the disease mitral regurgitationmitral regurgitation is always present. is always present.

There ise resting There ise resting pressure gradient at LV outflow tract in %35 of patients. pressure gradient at LV outflow tract in %35 of patients. Gradient can be precipitted at the other %25 (by augmenting myocardial Gradient can be precipitted at the other %25 (by augmenting myocardial

contraction, and reducing ventricular volume). contraction, and reducing ventricular volume). Fibrosis and occlusive disease in in small coronary arteries.Fibrosis and occlusive disease in in small coronary arteries.

The major coronary arteries, are wide and patent unless occlusive The major coronary arteries, are wide and patent unless occlusive atherosclerosis occurs. atherosclerosis occurs.

HCM:HCM:Symtomps:Symtomps: Angina, Dyspne, Presyncope, Syncope. Angina, Dyspne, Presyncope, Syncope.

Physical Findings: Physical Findings: 1-1- Before LV beat, LA beat is Before LV beat, LA beat is palpated (S4):palpated (S4): Is present even in the Is present even in the absence of any gradient or murmur. Impaired LV relaxation. absence of any gradient or murmur. Impaired LV relaxation.

2- LVOT Sistolik ejectıon Murmur:2- LVOT Sistolik ejectıon Murmur: Crescendo-Decrescendo, Crescendo-Decrescendo, starts with S1 and ends with S2.starts with S1 and ends with S2.

Best heard between apex and left sternal border. Cervical radiation is Best heard between apex and left sternal border. Cervical radiation is weak. Augmented by manouvers and drugs which decrease weak. Augmented by manouvers and drugs which decrease preload. (Valsalva, standing, amyl nitrite). Attenuates with preload. (Valsalva, standing, amyl nitrite). Attenuates with increasing afterload (squating, handgrip fenilefrin). increasing afterload (squating, handgrip fenilefrin).

3- 3- MR murmur:MR murmur: Heard at late systole, radiates to axilla, and Heard at late systole, radiates to axilla, and related with LV outflow obstruction. Mitral diastolic rumble and related with LV outflow obstruction. Mitral diastolic rumble and Paradoxic splitting of S2 may be heard. Paradoxic splitting of S2 may be heard.

4- Hyperdynamic carotis pulse.4- Hyperdynamic carotis pulse.

HCM: Clinical Presentation and MechanismsHCM: Clinical Presentation and Mechanisms

Chest pain:Chest pain: Ischemia, LVOT ob. Reduced coronary Ischemia, LVOT ob. Reduced coronary perfusion pressure.perfusion pressure.Exertional dyspnea:Exertional dyspnea: Diastolic dysfunction. Diastolic dysfunction.Reduced functional capacity:Reduced functional capacity: LVOTob, systolic LVOTob, systolic dysfunction, AFwith uncontrolled rapid ventricular dysfunction, AFwith uncontrolled rapid ventricular rate. rate. Palpitation:Palpitation: SVT, AF, frequent VPB, non-sustained SVT, AF, frequent VPB, non-sustained VT. VT. Syncope/Presyncope:Syncope/Presyncope: Supraventricular arrythmia, Supraventricular arrythmia, LVOTob, vasovagal, high VT rate .LVOTob, vasovagal, high VT rate .

İnadequate increase cardiac output during the effort. İnadequate increase cardiac output during the effort.

Cardiac arrest:Cardiac arrest: VT, SVT, AF, VF, bradyarrythmia. VT, SVT, AF, VF, bradyarrythmia.

HCM: Sudden death:HCM: Sudden death:

Supraventricular arrythmia is present in %20-50 of the Supraventricular arrythmia is present in %20-50 of the patients, but sudden death is caused by ventricular patients, but sudden death is caused by ventricular arrythmia. arrythmia.

Major Risk Factors of Sudden Death: Major Risk Factors of Sudden Death: 1-1- Patient who survive after cardiac arrest (sustained Patient who survive after cardiac arrest (sustained

ventricular tachycardia). ventricular tachycardia). 2- 2- Arrythmia and abnormal blood pressure response to Arrythmia and abnormal blood pressure response to

excersize.excersize.3-3- Non-sustained ventricular tachycardia (Holter). Non-sustained ventricular tachycardia (Holter). 4-4- Family history of sudden cardiac death and syncope in Family history of sudden cardiac death and syncope in

more than two first degree relatives before 40 years. more than two first degree relatives before 40 years. 5- 5- LV Wall thickness >30 mm. LV Wall thickness >30 mm.

HCM: Other manifestations.HCM: Other manifestations.

Atrial FibrillationAtrial Fibrillation

Seen in %15 of patients with HCM.Seen in %15 of patients with HCM.Absence of atrial systole. Rapid ventricular rate Absence of atrial systole. Rapid ventricular rate causes pulmonary edema or hypotension. causes pulmonary edema or hypotension. Rapid ventricular rateRapid ventricular rate causes causes detoriation of detoriation of functional capacity. functional capacity. By conversion to sinus rythm or decreasing heart By conversion to sinus rythm or decreasing heart rate functional capacity improves.rate functional capacity improves.

Endocarditis: Endocarditis: May occur on aortic or mitral valves. Unexpected heart May occur on aortic or mitral valves. Unexpected heart

failure and IE symptoms or signs should be failure and IE symptoms or signs should be suggest İE in HCM patiernts. suggest İE in HCM patiernts.

HCM: ECG and CHEST FİLMHCM: ECG and CHEST FİLM

Chest film:Chest film: May be normal-large left heart chambers. . No May be normal-large left heart chambers. . No aortic calcification. aortic calcification.

ECG:ECG: Is anormal in %97 of symptomatic HCMP, and in %90 Is anormal in %97 of symptomatic HCMP, and in %90 of asymptomatic HCMP patients. of asymptomatic HCMP patients. AFAF is detected in %15. is detected in %15. Non-sustained VTNon-sustained VT is frequent. . is frequent. . Q wavesQ waves in DII, DIII, aVF and D1, aVL, V5, V6 (and less in DII, DIII, aVF and D1, aVL, V5, V6 (and less frequently in V1-3). This sign shows hypertrophy, and frequently in V1-3). This sign shows hypertrophy, and causes pseudoinfarct patern. causes pseudoinfarct patern. Intraventricular conduction delay.Intraventricular conduction delay.

High voltageHigh voltage findings of findings of LVH. LVH. T waves of LVHT waves of LVH. . Huge negative T wavesHuge negative T waves are frequently seen in apical HCMP are frequently seen in apical HCMP

high precordial QRS voltage. high precordial QRS voltage. Short PR and pre-exitationShort PR and pre-exitation may be seen, but is may be seen, but is infrequent. infrequent.

HCMP: Echocardiographic HallmarksHCMP: Echocardiographic Hallmarks

Asymetric (dispropotionate) septal thickening: Asymetric (dispropotionate) septal thickening: Septum Septum to posterior wall ratio > 1.5to posterior wall ratio > 1.5

LV myocardial segment >1.5 cm in thicknesss.LV myocardial segment >1.5 cm in thicknesss.

Poor Septal contractıon. Hypercontractile free posterior Poor Septal contractıon. Hypercontractile free posterior wall.wall.

Systolic anterior motıon of the mitral valve (Systolic anterior motıon of the mitral valve (SAMSAM) when ) when outflow tract gradient >30 mmHg . outflow tract gradient >30 mmHg .

Mid-systolic closure of aortic valveMid-systolic closure of aortic valve. .

Small LV cavity.Small LV cavity.

Mitral regurgitationMitral regurgitation is frequent. is frequent.

LVOT gradient at rest present in about %35 of patientsLVOT gradient at rest present in about %35 of patients

HCM: Characteristic ECG paterns.HCM: Characteristic ECG paterns.

Left axis deviationLeft axis deviation

LBBBLBBB

Pathologic Q wave on anterolateral leads.Pathologic Q wave on anterolateral leads.

T wave inversion (commonly in İnferolateral leads)T wave inversion (commonly in İnferolateral leads)

ST segment changes.ST segment changes.

Criteria for left atrial enlargement.Criteria for left atrial enlargement.

V3-5 or V4-6 huge T wave inversion V3-5 or V4-6 huge T wave inversion (“Distal- apikal (“Distal- apikal HCM”HCM”

HCM:HCM: Pseudoinfarction patern, Q wave.Pseudoinfarction patern, Q wave.

HCMP: Management. Pharmacologic and HCMP: Management. Pharmacologic and

surgical intervention.surgical intervention. OBSTRUCTIVE OBSTRUCTIVE PHASE:PHASE:

Beta bockers:Beta bockers: (Especially (Especially latent obstruction). latent obstruction).

Verapamil. Verapamil. DisopiramidDisopiramid.. AmiodaronAmiodaron::

(SVT,(SVT, VAVA + + ).). Digoxin:Digoxin: ( (contraindicatedcontraindicated). ). Diüretic:Diüretic: ( (contraindicatedcontraindicated).).ACEIACEI ( (contraindicatedcontraindicated).).Surgery:Surgery: Septal myectomy. Septal myectomy.Septal ablation.Septal ablation.ICDICD is indicated in severe is indicated in severe

VA.VA.

LAST PHASE:LAST PHASE:Beta blockers:Beta blockers: (small doses) (small doses)Verapamil:Contraindicate.Verapamil:Contraindicate.Disopiramid:Contraindicate.Disopiramid:Contraindicate.Digoxin:Digoxin: Beneficial. Beneficial. Diuretic:Diuretic: Beneficial. Beneficial. ACEI:ACEI: Beneficial in patients Beneficial in patients

with LV dilatation and HF.with LV dilatation and HF.Surgery:Surgery: Transplantation. Transplantation.

HOCM: HOCM: LVOT gradient.LVOT gradient.

Hypertrophic obstructive cardiomyopathy (HOCM): Hypertrophic obstructive cardiomyopathy (HOCM): As As Mitral valve changes:When the LVOTis narrowed, blood rushes Mitral valve changes:When the LVOTis narrowed, blood rushes through the passageway toward the aortic valve(like tight garden through the passageway toward the aortic valve(like tight garden hose nozzle), dragging the leaflets of the mitral valve with it. Mitral hose nozzle), dragging the leaflets of the mitral valve with it. Mitral valve normally functıons keep blood floıwing in direction from the valve normally functıons keep blood floıwing in direction from the left atrium (upper heart chamber) to the LV. However increased left atrium (upper heart chamber) to the LV. However increased force of blood caused by HCM pulls the valve open and may cause force of blood caused by HCM pulls the valve open and may cause blood leak backward (called regurgitatıon )into the LA.blood leak backward (called regurgitatıon )into the LA.

NORMAL LVOTobs.

Anterıor replacement of the papillary muscle in HOCM : MR, SAM

HOCM: HOCM: Septal myectomy:Septal myectomy: Before the operatıon There is severe Before the operatıon There is severe hypertrophy of the basal septum, whith systolic anterior motıon of the mitral hypertrophy of the basal septum, whith systolic anterior motıon of the mitral

valve valve (-A-(-A-). This results in severe LVOT obs. as well as MR. During the ). This results in severe LVOT obs. as well as MR. During the surgery surgery (-B(-B ), yhe portıon of the basal septum that project into the outflow ), yhe portıon of the basal septum that project into the outflow

tract is removed by scalpel, resulting in abolitıon of the LVOTobs. tract is removed by scalpel, resulting in abolitıon of the LVOTobs. (-C-(-C-). There ). There is no longer SAM, and theMR abolished.is no longer SAM, and theMR abolished.

HOCM:HOCM: Septal ablationSeptal ablation (with absolute ethanol). (with absolute ethanol). İndication:İndication: LVOT gradient at rest > 30-50 mmHg. With LVOT gradient at rest > 30-50 mmHg. With

provocation 75-100 mmHg.provocation 75-100 mmHg.

HOCM:HOCM: Decreased LVOT gradient after septal ablation.Decreased LVOT gradient after septal ablation.