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

HCM: Symtomps: Angina, Dyspne, Presyncope, Syncope. Physical Findings: 1- Before LV beat, LA beat is palpated (S4): Is present even in the absence of any gradient or murmur. Impaired LV relaxation. 1- Before LV beat, LA beat is palpated (S4): Is present even in the absence of any gradient or murmur. Impaired LV relaxation. 2- LVOT Sistolik ejectıon Murmur: Crescendo-Decrescendo, starts with S1 and ends with S2. 2- LVOT Sistolik ejectıon Murmur: Crescendo-Decrescendo, starts with S1 and ends with S2. Best heard between apex and left sternal border. Cervical radiation is weak. Augmented by manouvers and drugs which decrease preload. (Valsalva, standing, amyl nitrite). Attenuates with increasing afterload (squating, handgrip fenilefrin). 3- MR murmur: Heard at late systole, radiates to axilla, and related with LV outflow obstruction. Mitral diastolic rumble and Paradoxic splitting of S2 may be heard. 3- MR murmur: Heard at late systole, radiates to axilla, and related with LV outflow obstruction. Mitral diastolic rumble and Paradoxic splitting of S2 may be heard. 4- Hyperdynamic carotis pulse. 4- Hyperdynamic carotis pulse.

HCM: Clinical Presentation and Mechanisms Chest pain: Ischemia, LVOT ob. Reduced coronary perfusion pressure. Chest pain: Ischemia, LVOT ob. Reduced coronary perfusion pressure. Exertional dyspnea: Diastolic dysfunction. Exertional dyspnea: Diastolic dysfunction. Reduced functional capacity: LVOTob, systolic dysfunction, AFwith uncontrolled rapid ventricular rate. Reduced functional capacity: LVOTob, systolic dysfunction, AFwith uncontrolled rapid ventricular rate. Palpitation: SVT, AF, frequent VPB, non-sustained VT. Palpitation: SVT, AF, frequent VPB, non-sustained VT. Syncope/Presyncope: Supraventricular arrythmia, LVOTob, vasovagal, high VT rate. Syncope/Presyncope: Supraventricular arrythmia, LVOTob, vasovagal, high VT rate. İnadequate increase cardiac output during the effort. İnadequate increase cardiac output during the effort. Cardiac arrest: VT, SVT, AF, VF, bradyarrythmia. Cardiac arrest: VT, SVT, AF, VF, bradyarrythmia.

HCM: Other manifestations. Atrial Fibrillation Seen in %15 of patients with HCM. Seen in %15 of patients with HCM. Absence of atrial systole. Rapid ventricular rate causes pulmonary edema or hypotension. Absence of atrial systole. Rapid ventricular rate causes pulmonary edema or hypotension. Rapid ventricular rate causes detoriation of functional capacity. Rapid ventricular rate causes detoriation of functional capacity. By conversion to sinus rythm or decreasing heart rate functional capacity improves. By conversion to sinus rythm or decreasing heart rate functional capacity improves.Endocarditis: May occur on aortic or mitral valves. Unexpected heart failure and IE symptoms or signs should be suggest İE in HCM patiernts.

HCM: ECG and CHEST FİLM Chest film: May be normal-large left heart chambers.. No aortic calcification. ECG: Is anormal in %97 of symptomatic HCMP, and in %90 of asymptomatic HCMP patients. AF is detected in %15. AF is detected in %15. Non-sustained VT is frequent.. Non-sustained VT is frequent.. Q waves in DII, DIII, aVF and D1, aVL, V5, V6 (and less frequently in V1-3). This sign shows hypertrophy, and causes pseudoinfarct patern. Q waves in DII, DIII, aVF and D1, aVL, V5, V6 (and less frequently in V1-3). This sign shows hypertrophy, and causes pseudoinfarct patern. Intraventricular conduction delay. Intraventricular conduction delay. High voltage findings of LVH. T waves of LVH. T waves of LVH. Huge negative T waves are frequently seen in apical HCMP high precordial QRS voltage. Short PR and pre-exitation may be seen, but is infrequent. Short PR and pre-exitation may be seen, but is infrequent.

HCM: Characteristic ECG paterns. Left axis deviation Left axis deviation LBBB LBBB 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 (“Distal- apikal HCM” V3-5 or V4-6 huge T wave inversion (“Distal- apikal HCM”

HCM: Pseudoinfarction patern, Q wave.

HCMP: Echocardiographic Hallmarks Asymetric (dispropotionate) septal thickening: Septum to posterior wall ratio > 1.5 Asymetric (dispropotionate) septal thickening: Septum to 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 wall. Poor Septal contractıon. Hypercontractile free posterior wall. Systolic anterior motıon of the mitral valve (SAM) when outflow tract gradient >30 mmHg. Systolic anterior motıon of the mitral valve (SAM) when outflow tract gradient >30 mmHg. Mid-systolic closure of aortic valve. Mid-systolic closure of aortic valve. Small LV cavity. Small LV cavity. Mitral regurgitation is frequent. Mitral regurgitation is frequent. LVOT gradient at rest present in about %35 of patients LVOT gradient at rest present in about %35 of patients

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

Anterıor replacement of the papillary muscle in HOCM : MR, Systolic anterior motion (SAM)

HCM vs Athlete’s Heart HCM Athlete HCM Athlete +Unusual pattern of LVH- +LV cavity 55 mm+ +LA enlargement- +Bizarre ECG paterns - +Abnormal LV filling - +Female gender - -  thickness with deconditioning + +Family history of HCM- Circulation 1995; 91:1596

HOCM: Septal myectomy: Before the operatıon There is severe hypertrophy of the basal septum, whith systolic anterior motıon of the mitral valve (-A-). This results in severe LVOT obs. as well as MR. During the surgery (-B ), yhe portıon of the basal septum that project into the outflow tract is removed by scalpel, resulting in abolitıon of the LVOTobs. (-C-). There is no longer SAM, and theMR abolished.

HOCM: Septal ablation (with absolute ethanol). İndication: LVOT gradient at rest > mmHg. With provocation mmHg.

HOCM: Decreased LVOT gradient after septal ablation.

Restictive Cardiomyopathy

Tanım: Tanım: Ventriküler sertliğin daha farklı mekanizma/patolojilerle artış gösterdiği bir grup bozukluğu temsil etmektedir. İnfiltrtif, inflamatuar veya metabolik hastalıkların sonucunda restriktif kardiyomyopati gelişebilir. Ventriküler sertliğin daha farklı mekanizma/patolojilerle artış gösterdiği bir grup bozukluğu temsil etmektedir. İnfiltrtif, inflamatuar veya metabolik hastalıkların sonucunda restriktif kardiyomyopati gelişebilir.

Idiopathic Dilated Cardiomyopathy

IDC - Definition a disease of unknown etiology that principally affects the myocardium a disease of unknown etiology that principally affects the myocardium LV dilatation and systolic dysfunction LV dilatation and systolic dysfunction pathology pathology increased heart size and weight increased heart size and weight ventricular dilatation, normal wall thickness ventricular dilatation, normal wall thickness heart dysfunction out of portion to fibrosis heart dysfunction out of portion to fibrosis

Incidence and Prognosis 3-10 cases per 100, cases per 100,000 20,000 new cases per year in the U.S.A. 20,000 new cases per year in the U.S.A. death from progressive pump failure 1-year25% 2-year35-40% 5-year40-80% death from progressive pump failure 1-year25% 2-year35-40% 5-year40-80% stabilization observed in 20-50% of patient stabilization observed in 20-50% of patient complete recovery is rare complete recovery is rare

Observed Survival of 104 Patients Years Am J Cardiol 1981; 47:525

Predicting Prognosis in IDC PredictivePossibleNot Predictive Clinical factorssymptomsalcoholismage peripartumduration family historyviral illness HemodynamicsLVEFLV size Cardiac indexatrial pressure DysarrhythmiaLV cond delayAV blocksimple VPC complex VPCatrial fibrillation Histologymyofibril volumeother findings Neuroendocrinehyponatremia plasma norepinephrine atrial natriuretic factor

Clinical Manifestations Highest incidence in middle age Highest incidence in middle age blacks 2x more frequent than whites blacks 2x more frequent than whites men 3x more frequent than women men 3x more frequent than women symptoms may be gradual in onset symptoms may be gradual in onset acute presentation acute presentation misdiagnosed as viral URI in young adults misdiagnosed as viral URI in young adults uncommon to find specific myocardial disease on endomyocardial biopsy uncommon to find specific myocardial disease on endomyocardial biopsy

History and Physical Examination Symptoms of heart failure Symptoms of heart failure pulmonary congestion (left HF) dyspnea (rest, exertional, nocturnal), orthpnea pulmonary congestion (left HF) dyspnea (rest, exertional, nocturnal), orthpnea systemic congestion (right HF) edema, nausea, abdominal pain, nocturia systemic congestion (right HF) edema, nausea, abdominal pain, nocturia low cardiac output fatigue and weakness low cardiac output fatigue and weakness hypotension, tachycardia, tachypnea, JVD hypotension, tachycardia, tachypnea, JVD

Cardiac Imaging Chest radiogram Chest radiogram Electrocardiogram Electrocardiogram 24-hour ambulatory ECG (Holter) 24-hour ambulatory ECG (Holter) lightheadedness, palpitation, syncope lightheadedness, palpitation, syncope Two-dimensional echocardiogram Two-dimensional echocardiogram Radionuclide ventriculography Radionuclide ventriculography Cardiac catheterization Cardiac catheterization age >40, ischemic history, high risk profile, abnormal ECG age >40, ischemic history, high risk profile, abnormal ECG

Clinical Indications for Endomyocardial Biopsy Definite Definite monitoring of cardiac allograft rejection monitoring of cardiac allograft rejection monitoring of anthracycline cardiotoxicity monitoring of anthracycline cardiotoxicity Possible Possible detection and monitoring of myocarditis detection and monitoring of myocarditis diagnosis of secondary cardiomyopathies diagnosis of secondary cardiomyopathies differentiation between restrictive and constrictive heart disease differentiation between restrictive and constrictive heart disease

Management Limit activity based on functional status Limit activity based on functional status salt restriction of a 2-g Na + (5g NaCl) diet salt restriction of a 2-g Na + (5g NaCl) diet fluid restriction for significant low Na+ fluid restriction for significant low Na+ initiate medical therapy initiate medical therapy ACE inhibitors, diuretics ACE inhibitors, diuretics digoxin, carvedilol digoxin, carvedilol hydralazine / nitrate combination hydralazine / nitrate combination

Management consider adding ß-blocking agents if symptoms persists consider adding ß-blocking agents if symptoms persists anticoagulation for EF <30%, history of thromboemoli, presence of mural thrombi anticoagulation for EF <30%, history of thromboemoli, presence of mural thrombi intravenous dopamine, dobutamine and/or phosphodiesterase inhibitors intravenous dopamine, dobutamine and/or phosphodiesterase inhibitors cardiac transplantation cardiac transplantation

myocardial noncompaction myocardial noncompaction