Kuliah S1

Kardiomiopati

Departemen Kardiologi dan Kedokteran Vaskular,

Fakultas Kedokteran Universitas Indonesia

Tipe kardiomiopati
WHO mengklasifikasi berdasarkan anatomi
dan fisiologi :
Kardiomiopati dilatasi
Kardiomiopati hipertrofi
Kardiomiopati Restriksi

Kardiomiopati Dilatasi
Etiologi :
Peripartum
Diabetes
Iskemia
Infeksi : virus, bakteri
Insufisiensi vit B 1
Sistemik / Imunologis
Tidak diketahui

Kardiomiopati Dilatasi
Definisi
Kelainan jantung yang ditandai oleh dilatasi
salah satu atau kedua ventrikel disertai
disfungsi sistolik dan diastolik

Patogenesis Kardiomiopati
Dilatasi
Remodeling jantung
Respon kompensasi maladaptasi terhadap disfungsi kontraksi
dan peningkatan volume ventrikel kiri
Elongasi kardiomiosit
Dikontribusi oleh aktivasi neurohormonal

Aktivasi simpatis
Peningkatan aktivasi simpatis mekanisme kompensasi
Down regulation dari reseptor

Sistem Renin Angiotensin

Angiotensin I angiotensin II oleh ACE
Angiotensin II remodeling jantung

Presentasi Klinis
Dilatasi kardiomiopati
Gejala dan tanda tidak spesifik
Presentasi sesuai gagal jantung
Gagal jantung kiri
Gagal jantung kanan
Gagal jntung kongestif

Embolisasi stroke

Pendekatan diagnosis
Anamnesis : sesuai gagal jantung
Pemeriksaan Fisik : bisa sesuai gagal jantung
Radiologis :
CXR melebar
Bendungan paru

EKG :
LVH voltage

Ekokardiografi :
Dilatasi ruang jantung
disfungsi sistolik dan disfungsi diastolik

Penatalaksanaan dan terapi

Terapi simtomatik
Diuretik
ACE inhibitor
Beta bloker

Kardiomiopati Hipertrofi
Definisi
Kelainan jantung yang ditandai oleh hipertrofi
miokardial tanpa diketahui etiologinya.
Hipertrofi dengan atau tanpa obstruksi.

Beberapa terminologi :
Hypertrophic Obstructive Cardiomyopathy (HOCM)
Hypertrophic Cardiomyopathy (HCM)
Idiopatic Hypertrophic Subaortic Stenosis
Asymmetrical Septal Hypertrophy

Kardiomiopati Dilatasi
Familial autosomal dominan
Insidens tertinggi : usia remaja, dekade 4 dan
dekade 6
Terutama laki-laki pada usia muda
Terutama perempuan pada usia tua

Presentasi Klinis
Kardiomiopati Hipertrofi

Asimtomatik
Paling sering : sesak , sakit dada, pingsan
Aritmia
Sudden death
Varian : hipertrofi apikal

Pendekatan diagnostik
Anamnesis
Pemeriksaan fisik
Murmur sistolik ejeksi di area LSB
Murmur sistolik di area mitral

Radiologi
Tidak ada tanda khas

EKG
Abnormalitas segmen ST dan gelombang T
LVH

Ekokardiografi
LVH
Disfungsi diastolik tapi fungsi sistolik normal

Penatalaksanaan dan terapi

Medikal
Menangani gejala obat inotropik negatif, obat pilihan : beta
bloker
Hindari keadaan hipovolemi atau yang membuat hipekinetik

Intervensi :
Pacu jantung permanen
Implant Defibrilator Kardioversi
ablasi alkohol di septum ventrikel

Pembedahan
Subaortic miotomi , reseksi basal septum
Koreksi katup mitral (kalau ada MR)
Transplantasi jantung

Prediktor penderita HCM dengan

risiko tinggi
Riwayat cardiac arrest
Ventrikel takikardi
Riwayat keluarga dengan sudden death
Non sustained VT ditemukan dari pemeriksaan Holter

Sinkope atau presinkope bukan neurogenik

LVOT gradient > 50 mmHg

Tebal dinding LV > 20 mm
Dilatasi LA > 45 mm
Respons hipotensi saat exercise

Definition
Restrictive cardiomyopahty
Characterized by restrictive filling and reduce
diastolic volume either or both ventricles with
normal or near normal systolic function and wall
thickness

Circulation 1996;93:841-842

N Engl J Med 2003;348:1639-46

Classification of restrictive
cardiomyopathies

(Curr Probl Cardiol 2004;29:503-67.

Clinical feature
Clinical manifestation may vary depending
on whether the left, right or both ventricles
are involved in the process.
Complain of respiratory, followed by
abnormal physical examination, heart failure
and syncope ( Shannon et al .2000)

Clinical feature

Circulation 2000;101:2490-2496

Investigation
ECG ( lewis, 1992 )
Shows atrial enlargement in virtual
patient.
LVH is seen in less than half
Impaired AV conduction is frequently
reported
Chest radiography
CTR greater than 0.5 ( lewis,1992)
Pulmonary venous congestion is a

Echocardiography

Patologi anatomi

Investigation

Investigation
Cardiac catheterization
required to evaluate the pulmonary vascular

resistance because it may be

markedly elevated even at
presentation.
The pulmonary vascular resistance
can be rise
markedly within month to several
years of the
initial diagnosis*
left ventricular end-diastolic pressure is usually

*Tex Heart Inst J 1997;24:38-44;**Cardiology in review 2002;10(4):210-229

Prognosis
The probable outcome is poor, and
treatment is not usually very effective.
People with restrictive cardiomyopathy
may be candidates for heart transplant

Prognosis
Median survival was 1.4 years,
six patient from eight died 0.2 to 7.0 years
after they were initially seen*
Embolic events occurred in 33%**
Markers for poor prognosis for sudden
death with presentation sign or symptom
ischemia***
*Am Heart J.1992 Jun;123(6):1589-93;**Tex
Heart Inst J 1997;24:38-44;***Circulation 2000;102:876-882
Jun;123(6):1589-93;

Definition
Constrictive pericarditis
Result from a thickened, scarred and often
calcified pericardium that limits diastolic
ventricular filling

Circulation 2002;105:2939-2942

Heart 2001;86:343-349

Clinical feature
The chronic form
Usually present with ascites,
hepatomegaly,
dyspnea and failure to thrive. Symptoms
are
usually present for a months to years
before
correct diagnosis is made
The sub acute form
HEART 2001;86:343-349
Follows an acute infectious illness by day

Management and Prognosis

Pericardiectomy is the definitive treatment
for constrictive pericarditis in severe
constrictive with mortality 6 % to 19%

Circulation 2002;105:2939-2942

Constriction vs. Restriction

Curr Probl Cardiol 2004;29:503-67

Constriction vs. Restriction

Curr Probl Cardiol 2004;29:503-67

Curr Probl Cardiol 2004;29:503-67