Kuliah S1 Kardiomiopati - Website Staff...
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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
• Definisi
Kelainan jantung yang ditandai oleh dilatasi salah satu atau kedua ventrikel disertai disfungsi sistolik dan diastolik
Kardiomiopati Dilatasi
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 KlinisDilatasi 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
• 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 Hipertrofi
Familial autosomal dominan
• Insidens tertinggi : usia remaja, dekade 4 dan dekade 6
• Terutama laki-laki pada usia muda
• Terutama perempuan pada usia tua
Kardiomiopati Dilatasi
Presentasi KlinisKardiomiopati 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;348:1639-46N 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 byabnormal 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 halfImpaired AV conduction is frequently
reported
• Chest radiographyCTR greater than 0.5 ( lewis,1992) Pulmonary venous congestion is a
common finding( cetta, 1995)
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 significantly higher (>5 mm Hg) than
right ventricular end-diastolic pressure.**
*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;*Am Heart J.1992 Jun;123(6):1589-93;**Tex Heart Inst J 1997;24:38-44;***Circulation 2000;102:876-882
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
Follows an acute infectious illness by day to
several month
HEART 2001;86:343-349
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