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

left ventricular end-diastolic pressure is usually

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