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