Kardiomiopati - Website Staff UI

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 ß
• 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
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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