KARDIOMYOPATIKARDIOMYOPATI

dr. Erlina Marfianti, MSc, SpPDdr. Erlina Marfianti, MSc, SpPD

Dept. Ilmu Penyakit Dalam FK UII YK Dept. Ilmu Penyakit Dalam FK UII YK

DefinisiDefinisi

Suatu kelompok penyakit yang mengenai Suatu kelompok penyakit yang mengenai langsung pada otot jantung itu sendiri, langsung pada otot jantung itu sendiri, yang bukan terjadi karena kondisi lain yang bukan terjadi karena kondisi lain akibat perikarditis, koroner, hipertensi, akibat perikarditis, koroner, hipertensi, penyakit kongenital, penyakit katup.penyakit kongenital, penyakit katup.

Menyebabkan disfungsi / kehilangan Menyebabkan disfungsi / kehilangan kemampuan untuk kemampuan untuk

EtiologiEtiologi

Kebanyakan kasus adalah idiopatikKebanyakan kasus adalah idiopatik Merupakan 30 % penyebab kematian jantungMerupakan 30 % penyebab kematian jantung Perkembangan ilmu Perkembangan ilmu genetik genetik Berdasarkan etiologi diklasifikasikan menjadi 2 Berdasarkan etiologi diklasifikasikan menjadi 2

tipe:tipe: Tipe primer : bila penyebabnya tidak diketahuiTipe primer : bila penyebabnya tidak diketahui (idiopatik kardiomiopati, familial kardiomiopati)(idiopatik kardiomiopati, familial kardiomiopati) Tipe skunder: bila diketahui ada penyakit miokardium Tipe skunder: bila diketahui ada penyakit miokardium

yang penyebabnya tidak diketahuiyang penyebabnya tidak diketahui

KlasifikasiKlasifikasi Berdasarkan klinis dan patologis dibagi Berdasarkan klinis dan patologis dibagi

menjadi 3, yaitu:menjadi 3, yaitu: Kardiomiopati hipertrofikKardiomiopati hipertrofik Kardiomiopati RestriktifKardiomiopati Restriktif Kardiomipati DilatasiKardiomipati Dilatasi

Dilated (congestive) cardiomyopathyDilated (congestive) cardiomyopathy

TThis is the most common form. In it, the heart his is the most common form. In it, the heart cavity is enlarged and stretched (cardiac cavity is enlarged and stretched (cardiac dilation). dilation).

The heart is weak and doesn't pump normally, The heart is weak and doesn't pump normally, and most patients develop heart failure. and most patients develop heart failure.

Abnormal heart rhythms called arrhythmias and Abnormal heart rhythms called arrhythmias and disturbances in the heart's electrical conduction disturbances in the heart's electrical conduction also may occur.also may occur.

PenyebabPenyebabPaling sering: penyebabnya tidak diketahui,

• genetik

• Autoimun disease, amiloidosis, sarkoidosis, DM

SYMPTOMS OF CARDIOMYOPATHYSYMPTOMS OF CARDIOMYOPATHY

Shortness of breathShortness of breath Swelling of the anklesSwelling of the ankles TirednessTiredness Palpitations and faintingPalpitations and fainting Chest painChest pain

Physical examination: Physical examination: Cardiomegaly, S3 gallop, a murmur Cardiomegaly, S3 gallop, a murmur fucntional mitral regurgitation.fucntional mitral regurgitation.

DiagnosisDiagnosis

Electrocardiogram (ECG)Electrocardiogram (ECG) • • EchocardiogramEchocardiogram • • Chest X-rayChest X-ray • • Holter Monitor or 24 hour tapeHolter Monitor or 24 hour tape • • Exercise testExercise test • • Blood testsBlood tests Cardiac CateterizationCardiac Cateterization Biopsy myocardialBiopsy myocardial

The ECG may show changes that indicate heart muscle damage, but not necessarily the cause of this damage. In DCM the ECG abnormalities are not specific and may also be found in other conditions.

Figure 3Electrocardiogram or ECG

• Low QRS Voltage

•Non spesific repolarisation

•Intraventrikular conduction abnormalities

Echocardiogram or echo

An echocardiogram is an ultrasound scan of the heart (figure 4). A picture of the heart is produced from which an accurate assessment of the size and function of the heart can be made. This is the most important test in DCM as it confirms the presence of an enlarged and poorly functioning heart.

                                                                                                                                                              

Figure 4Echo machine

TreatmentTreatment Spesific therapy underlying causedSpesific therapy underlying caused Sesuai heart failure:Sesuai heart failure:

ACE Inhibitors (such as Captopril, Enalapril, Lisinopril, Enalapril ACE Inhibitors (such as Captopril, Enalapril, Lisinopril, Enalapril and others).and others).

Beta-blockers (such as Metoprolol, Carvedilol, Bisoprolol and Beta-blockers (such as Metoprolol, Carvedilol, Bisoprolol and others) others)

DigoxinDigoxin Diuretics (such as Frusemide, Bumetamide, Amiloride and others)Diuretics (such as Frusemide, Bumetamide, Amiloride and others) SpironolactoneSpironolactone WarfarinWarfarin AmiodaroneAmiodarone

Other therapiesOther therapies

CardioversionCardioversion Heart transplantHeart transplant PacemakerPacemaker

COMPLICATIONSCOMPLICATIONS

Heart failureHeart failure This is a common feature of DCM. It occurs when the muscle of the heart is This is a common feature of DCM. It occurs when the muscle of the heart is

not strong enough to pump blood efficiently round the body, causing build not strong enough to pump blood efficiently round the body, causing build up of fluid in the lungs and /or the tissues.up of fluid in the lungs and /or the tissues.

Atrial fibrillation (AF)Atrial fibrillation (AF) This is a very common abnormal rhythm in DCM. The heart beat is irregular This is a very common abnormal rhythm in DCM. The heart beat is irregular

and rapid, and causes palpitations, increasing shortness of breath and and rapid, and causes palpitations, increasing shortness of breath and fatigue. It can be associated with sudden deterioration of symptoms or the fatigue. It can be associated with sudden deterioration of symptoms or the development of blood clots (emboli). The risk of clots means that warfarin development of blood clots (emboli). The risk of clots means that warfarin medication is usually used to thin the blood if atrial fibrillation occurs.medication is usually used to thin the blood if atrial fibrillation occurs.

   Blood clotsBlood clots In DCM the blood flow through the heart is slower than normal. This may In DCM the blood flow through the heart is slower than normal. This may

allow blood clots to form in the heart. If a blood clot dislodges from these allow blood clots to form in the heart. If a blood clot dislodges from these sites and goes into circulation, it can cause damage to the brain (a stroke). sites and goes into circulation, it can cause damage to the brain (a stroke). Therefore, some people with DCM ,especially those with very enlarged Therefore, some people with DCM ,especially those with very enlarged hearts, may need to be treated with anticoagulant medication (warfarin), to hearts, may need to be treated with anticoagulant medication (warfarin), to prevent such clots from forming.prevent such clots from forming.

Rhythm disordersRhythm disorders These generally cause dizziness, These generally cause dizziness,

breathlessness, palpitations (awareness of the breathlessness, palpitations (awareness of the heartbeat), or may cause no symptoms. Some heartbeat), or may cause no symptoms. Some rhythm disorders that may occur in DCM are:rhythm disorders that may occur in DCM are:

Ventricular ectopicsVentricular ectopics Ventricular tachycardia (VT)Ventricular tachycardia (VT) Ventricular fibrillation (VF)Ventricular fibrillation (VF) Sudden deathSudden death Heart blockHeart block

hypertrophic cardiomyopathyhypertrophic cardiomyopathy

hypertrophic cardiomyopathyhypertrophic cardiomyopathyIn this condition, the muscle mass of the left In this condition, the muscle mass of the left ventricle enlarges or "hypertrophies."ventricle enlarges or "hypertrophies."

In one form of the disease, the wall (septum) In one form of the disease, the wall (septum) between the two ventricles (pumping chamber) between the two ventricles (pumping chamber) becomes enlarged and obstructs the blood flow becomes enlarged and obstructs the blood flow from the left ventricle. The syndrome is known from the left ventricle. The syndrome is known as hypertrophic obstructive cardiomyopathy or as hypertrophic obstructive cardiomyopathy or asymmetric septal hypertrophy asymmetric septal hypertrophy 

It's also called idiopathic hypertrophic subaortic It's also called idiopathic hypertrophic subaortic stenosis stenosis

Symptoms

Chest pain Dizziness

Fainting, especially during exercise Heart failure (in some patients)

High blood pressure (hypertension) Light-headedness, especially after activity or exercise

Sensation of feeling the heart beat (palpitations) Shortness of breath

Other symptoms that may occur are:Fatigue, reduced activity tolerance

Shortness of breath when lying down

Some patients have no symptoms. They may not even realize they have the condition

until it is found during a routine medical exam.

DiagnosisDiagnosis Tests used to diagnose heart muscle thickness, problems with blood Tests used to diagnose heart muscle thickness, problems with blood

flow, or leaky heart valves (flow, or leaky heart valves (mitralmitral valve regurgitation valve regurgitation) may include:) may include: 24-hour 24-hour HolterHolter monitor monitor (heart monitor) (heart monitor) Cardiac catheterization Cardiac catheterization Chest x-ray Chest x-ray ECG ECG Echocardiography (the most common test) with Doppler Echocardiography (the most common test) with Doppler

ultrasound ultrasound MRI of the heart MRI of the heart Transesophageal echocardiogram (TEE) Transesophageal echocardiogram (TEE) Blood tests may be done to rule out other possible diseases.Blood tests may be done to rule out other possible diseases. If you are diagnosed with hypertrophic cardiomyopathy, your health If you are diagnosed with hypertrophic cardiomyopathy, your health

care provider may recommend that your close blood relatives care provider may recommend that your close blood relatives (family members) be screened for the condition(family members) be screened for the condition

TreatmentTreatment

The goal of treatment is to control symptoms and prevent complications. Some patients may need to stay in the hospital until the condition is under control (stabilized).If you have symptoms, you may need medication to help the heart contract and relax correctly. Drugs include beta-blockers and calcium channel blockers. These medicines reduce chest pain and pain during exercise. Medications will often relieve symptoms so patients do not need more invasive treatments.Some people with arrhythmias may need anti-arrhythmic medications. If the arrhythmia is due to atrial fibrillation, blood thinners will also be used to reduce the risk of blood clots.Some patients may have a permanent pacemaker placed. However, pacemakers are used less often today than they were in the past.

Restrictive cardiomyopathy is a disorder of the heart muscle in which the walls of the ventricles become stiff, but not necessarily thickened, so they resist normal filling with blood.

Impaired diastolic filling with preserved contractile function

EtiologiEtiologi idiopathicidiopathic (its cause isunknown) (its cause isunknown) systemic disorderssystemic disorders such as: such as:

endomyocardial fibrosis (tropical, hypereosinophic syndrome),endomyocardial fibrosis (tropical, hypereosinophic syndrome), infiltrative disorders (amyloidosis, sarcoidosis), andinfiltrative disorders (amyloidosis, sarcoidosis), and rare metabolic disorders (Gaucher's disease, rare metabolic disorders (Gaucher's disease,

Mucopolysaccharidoses, Fabry's disease, carcinoid syndrome). Mucopolysaccharidoses, Fabry's disease, carcinoid syndrome). Restrictive cardiomyopathy is also described following Restrictive cardiomyopathy is also described following

radiation therapyradiation therapy for some types of cancer. for some types of cancer. Patients with the idiopathic form may Patients with the idiopathic form may have a family have a family

historyhistory of cardiomyopathy. Recent evidence suggests of cardiomyopathy. Recent evidence suggests that the disease may be caused by the same genetic that the disease may be caused by the same genetic abnormalitiesabnormalities

Restrictive CardiomyopathyRestrictive CardiomyopathyThis is the least common type in the United States.

The myocardium (heart muscle) of the ventricles becomes excessively "rigid," so it's harder for the ventricles to fill with blood between heartbeats.

A person with restrictive cardiomyopathy often complains of being tired, may have swollen hands and feet, and may have difficulty breathing on exertion.

This type of cardiomyopathy is usually seen in the elderly and may be due to another disease process.

SymptompSymptomp

Patients with RCM have signs and/or symptoms Patients with RCM have signs and/or symptoms of heart failure such as of heart failure such as fatigue, shortness of fatigue, shortness of breath, tissue swelling (oedema) and abdominal breath, tissue swelling (oedema) and abdominal enlargementenlargement. .

Up to a third of patients may have an embolic Up to a third of patients may have an embolic complication, such as a blood clot. complication, such as a blood clot. 

Abnormal heart rhythms and palpitations are Abnormal heart rhythms and palpitations are common whatever the underlying cause of the common whatever the underlying cause of the disease disease

DiagnosisDiagnosis

The diagnosis of RCM is usually based on a The diagnosis of RCM is usually based on a physical physical examinationexamination, an , an electrocardiogram (ECG)electrocardiogram (ECG) and an and an echocardiogramechocardiogram. .

Magnetic resonance imaging (MRI) can provide Magnetic resonance imaging (MRI) can provide additional information about the structure of the heart.additional information about the structure of the heart.

In some patients a precise diagnosis may require In some patients a precise diagnosis may require catheterisation of the heart to measure pressures andcatheterisation of the heart to measure pressures and

a biopsy of the heart muscle (removal and microscopic a biopsy of the heart muscle (removal and microscopic examination of a specimen), which may enable the examination of a specimen), which may enable the doctor to identify the infiltrating substance.doctor to identify the infiltrating substance.

TreatmentTreatment

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