COR PULMONALE

Dr. S.E.E. Palupi Sp.JP

Cor Pulmonale chronicumHipertrofi dilatasi ventrikel kanan akibat hipertensi pulmonal yg diakibatkan oleh :•Penyakit paranchym paru.•Penyakit sistem vaskuler dari MPA – V. Pulmonalis.•Kelainan dinding thorax•Kelainan ventilasi paru•Kelainan dinding thorax.Disingkirkan :

Disfungsi ventrikel kanan (RV) akibat gagaljantung kiri.Congenital HD.M.S

• Cor pulmonale akutMeningkatnya beban R.V. secara akut akibat :Hipertensi pulmonal akut akibat emboli pulmonal pd thrombo emboli

Perjalanan Penyakit

Gagal jantung kanan dapat terjadi akut pd DVT (Deep Vein Thrombus emboli lepas

A. Pulmonalis dan cabang-cabangChronic : P.P.O.K.Akut on chronic : P.P.O.K dng infeksi atau

hypoxia beratUS 20% pend. Dng gagal jantung : C.Pulm.INDONESIA ? Insiden perokok

polusi

Fungsi Sirkulasi Pulmonal (normal)

• Pertukaran gas• Membuang particles• Konversi AI AIIRespiratory movement (bernafas)

memfasilitasi aliran darahInsp venous return Exp Peredaran sistemik

Stroke Volume R.V (= LV) tergantung :

• Preload• Kontraktilitas• Afterload• Patofisiologi :• Kenaikan afterload hypertrophy dan

dilatasi R.V.

Sebab-sebab Kenaikan Afterload• Hiper inflasi pd PPOK kompresi kapiler alveoli,

memanjangnya pembuluh darah paru.• Volume paru berkurang : post reseksi paru yg

luas. Penyakit paru restriktif kompresi dan distorsi pembuluh darah paru

• Hipoxia vasokontriksiHipertensi pulmonal

• Hipoxia Hematocrite • viskositas darah hipertensi pulmonal

A. Penyakit Vaskuler Paru

(Cor Pulm. Akibat penyakit vaskuler paru chronic)•Emboli pulm berulang•Recurrent medium sized emboli yg tdk mengalami lysis, organized chronic thrombo embolic pulm. hypertension.•Partikel yg berasal IVDA, metastase tumor.•Pulm. vasculitis•Pulm vasoconstriction pd dataran tinggi.•Primary Pulm. Hypertension.

Manifestasi Klinis :

• Dysp + tachypnea – Hip. Pulm.e.c. vasc.• Exercise dan istirahat• Tidak berkurang pd posisi duduk• Batuk tdk produktif• Chest pain : Dilatasi A. Pulmonalis atau

Ischemia R.V.• Hepatomegali, udema tungkai• Cyanosis : hypoxemia arterial atau

CO• R.V kuat angkat – para sternal kiri atau

epigastrium

• Ejection click• P2 keras• Proto diastolic gallop (S3), (S4)• Pan syst. Murmur ec TR.• JVP hepoto jugular reflux +• Kelainan fisik akibat R.V. failure dpt

berkurang/hilang apabila tekanan A. Pulmonalisdng menurunkan keadaan hipoxia

Laboratorium• Radiologi : A. Pulm. Dan cabang-cabang

dilatasi• Right Desc.Pulm. A>.• Adanya DVT menetapkan diagnosa Pulm.

Vasc. Disc (Vacular Doppler)• EKG : P.Pulmonale

– RVH– RAD– Aritmia – (mungkin)

• Echo : - Pengukuran tebal dinding RV - Dilatasi RV dibanding LV.

IVS : terdorong ke kiriMRI : mengukur massa RVMyocardial perfusion scintigraphyThallium scanningSestamibi RVH dpt diterapkan Normal : RV”is not imaged” uptake LV sangat

besarKateerisasi jantung

Tekanan vaskuler paruLung biopsi vaskulitis, dsb

II. Acute Cor Pulmonale akibat

Emboli massive atau MultipleAcute R.V failure akibat emboli paru massive

dicurigai pd hal sebagai berikut:• Sudden onset of severe dyspnoe• Cardio vascular collaps pd penderita dng

venous thrombosis.

Manifestasi Klinis Acute R.V. Failure

• Pucat • Berkeringat• Hypotension• Rapid pulse, small amplitude• Neck vein : distended.

V. wave nyata/ TR.• Hepatomegali : lunak

Nyeri tekanPulsatile

• Pan SM – TRPunct max : para sternal kiri

• S4 gallop• A.G .D : PO2 (Kegagalan ventilasi-perfusi)

PCO2 (hiperventilasi)

Terapi V.T.E.• LMWHMempunyai kelebihan dibanding unfractionated heparin :Tak perlu monitoring laboratorium (APTT)Pemberian s.c.Bio availability lebih tinggiPlasma half life lebih lamaL.O.S lebih pendek• UF heparinBolus : 60 – 70 U/kg, BB : max 5000 U.Dilanjutkan dengan:12 – 15 U/kg/jam max : 1000 U/jam.APTT 1,5 – 2,5 ><1controlHitung thrombocyt : mewaspadai induced

thrombocytopenia

• Oral anticoagulant (warfarin)3 – 6 bulan : reversible VTE:SurgeryTraumaPemakaian estrogen≥6 bulan : idiofathic12 bulan : - life time :Anti thrombin deficiency.• Obat-obat inotropicDobutamine infuse :Dosis : 2,5 – 10 mcg/kg/menitTitrasi : berdasar respon terhadap H.R.C.OUrine output

IndikasiTanda-tanda CO (vasocontriction) tetapi tekanan darah masih baik (SBP≥ 90 mmHg.Dopamine infuse:Dosis : 2,5 – 10 mcg/kg/menit

Titrasi berdasar : H.R, CO, Urine output

•O2Inhalasi : O2 100% menurunkan R.V

Afterload•Terapi thrombolyticIndikasi : massive P. embolism.Kontraindikasi : absolute

relative

Absolute C.I1. Active internal bleeding2. Recent spontaneous intra cranial bleedingRelative C.I1. Major surgeryDeliveryOrgan biopsyPuncture of a non compressible vessel in the preceding

10 days.2. IschStroke – 2 mo3. G.I bleeding – 10 days.4. Serious trauma – 15 days.5. Neuro surgery w/in 1 monthOpthalmic surgery

6. Uncontrolled severeHypertensionSBP ≥ 180 mmHg, DBP ≥ 110 mmHg7. Recent CPR8. Platelet count < 100.000/ml9. Pregnancy10. Bacterial endocarditis11. Diabetic hemorrhage retinopathy.Streptokinase :250.000 i.u IV loading dilanjutkan dengan100.000 i.u/jam – 24 jamTerapi thrombolytic diharapkan berhasil dng fragmentasi

embolus dan tekanan menurun cepat

ACUTE COR PULMONALE

Thrombi in right heart (septal infarction, atrial fibrillation)

Tumor emboli

Air emboli

Pelvic-vein thrombi (following pelvic or prostatic surgery)

Fat emboli (fractures)

Thrombi in deep veins of lower extremity

Multiple emboli in pulmonary arterial tree with acute infarcts

Massive emboli of pulmonary trunk and main pulmonary arteries without infarction; right heart dilated

Thrombus in Left common, external, and internal iliac

veins, loosely attached to the vessel wall; A common

source of pulmonary emboli

B. Cor Pulmonale Akibat Penyakit Parenchym Paru

Cor. Pulm. Pd PPOK menunjukkan prognosis yg kurang baik. Penyebab tersering PPOK.

Masalah Hipertensi Pulm pd PPOK

1. Pulm. Vasocontriction akibat hypoxiaStimuli menyebabkan Penebalan tunica media A. Pulm dan cabang-cabang.

2. Hilangnya pembuluh darah kecil, pd tempat emphysema, jaringan paru yg rusak.

3. Kenaikan CO & viskositas darah akibat polycythemia akibat hypoxia.

4. PPOK chronic hypoxia disfungsi LVtekanan LA

Tekanan A. PulmonalisDisfungsi LV akibat isch. Myocard pd perokok.

5. RV. FailureRV failure merupakan komplikasi cor pulmonale.Dicetuskan : gagal nafasInfeksi jalan nafasHipertensi pulm.6. Aritmia supraventricular dan atauVentricular7. Hepatomegali : lunak (palp)Tepi tumpulLicinHJR reflux +8. Udema perifer.Eksaserbasi obstruksi jalan nafas oleh infeksi tekanan

intrathoracic venous return udema perifer.9. Bendungan vena sistemik akibat obstr jalan nafas dpt menurun

apabila berkurangnya/ hilangnya obstr atau infeksi

Patologi Anatomi RVH, MPA dilatasi. Tunica media cabang-cabang A. Pulm menebal,

fibrosis, elastisitas sampai ke arteriole berkurang. Kapiler: Distorsi. dan Menghilang pd tempat-tempat Terjadi hiperinflasi paruManifestasi Klinis.Riwayat batuk produktifSesak nafas membatasi aktivitasRiwayat perawatan RS akibat ;Infeksi jalan nafasMech ventilation selama perawatanHypoxia pd malam hari akibat hypoventilation.

Pemeriksaan Fisik• Nicotine staining• Kulit hangat• Arterial pulse• Distensi thorax• Rhonchi +• Wheezing akibat bronchitis chs• S3 gallap dari RV• Pan SM TR• Udema perifer• Kenaikan systemic venous pressureAF, infeksi pulmonal.• Hepatomegali ; HJR (+) JVP

LaboratoriumFungsi pulmonal menurunFoto thoraxAkibat hiperinflasiPembesaran R.V sukar ditentukan MPA : dilatasi ke periferHilangEKG : Kurang peka menunjukkan RV hiperinflasi paru

konduktor buruk utk impuls listrik Aritmia:Echo : hipertrofi RV Dilatasi RVMyocardium perfusion scintigraphy RV uptake tinggi

Kateterisasi :Pulm. Artery Wedge Pressure masih mungkin normal

apabila tdk ada komplikasi.Theraphy.Harus optimal.Infeksi jalan nafas akibat akut yg dpt merupakan pemicu

RV failure. Harus segera diobati dan adequate.Memperbaiki ventilasiObstruksi diperbaikiO2 ditambahLong term O2 therapy dpt menelong penderita PPOK

tekanan A. Pulm.Broncho dilator dan A.B memperbaiki obstr jalan nafas.

Diuretica : Menghilangkan udemaIngat : venous return dan CODigitalis : RV failure.Mudah intoxicatie pd keadaan HypoxiaPhlebotomy : apabila Ht > 55 %Inhalasi NO – dlm evaluasi

C. Cor Pulm. Akibat Penyakit Paru RestrictiveObliterasi vascular bed.D. Cor Pulm. Akibat kelainan Vantilasi :• Neuromusc apparatus• Chest wall• DiaphragmaMech : Hypoxia chronicTertekannya cabang-cabang A. Pulm

E. Cor Pulm. Kel. Kontrol PernafasanSleep apneaObstruksi jalan nafas atasF. Cor Pulm. Pd KetinggianHypoxia chronic sec polycythemiaHipertensi Pulm.

Cor Pulm.Turun kedataran rendah

Hypoxia atau (-)

CHRONIC COR PULMONALE

Extensive pulmonary emphysema with great distention of pulmonary trunk and main pulmonary arteries which have pressed the aorta against the trachea; pulmonary arteriosclerosis and right ventricular hypertrophy

Hypertrophy and dilatationof right ventricle

X-Ray : Chronic obstructive emphysemawith cor pulmonale

R waves in leads V1 and V2 as well as S waves in leads I, V4, V5, and V6 are indicative of right ventricular hypertrophy; prominent P waves in leads II, III, aVF, V1 and V2 suggest right atrial enlargement

CHRONIC COR PULMONALE

Chronic Lung Disease

Reduction in pulmonary vascular bed

Acidosis and hypercapnia

Pulmonary hypertension

Polycythemia andhyperviscosity

Hypertrophy and dilatation of the right ventricle

Right ventricle failure

Hypoxia

WR Summer, in LJ Rubin (cd): Pulmonary Heart Disease, Boston, Martinus Nijhoff, 1984, P 285

Pathogenesis of Cor Pulmonale

Mechanism Responses Characteristic

RESPIRATORY DISEASE

Obstructive

Chronic bronchitis and emphysema : chronic asthma

Pulmonary hypertension due to hypoxia, vascular stretching, and loss of vessels. Heart beat impeded externally by lung hyperinflation. Normal or high output

Chronic cor pulmonale “Blue bloater” or “Pink puffer”

Restrictive

1. Intrinsic : interstitial fibrosis lung resection

Hypertension due to hypoxia, vascular distortion and loss.

Chronic cor pulmonale

Breathlessness

Hyperventilation

2. Extrinsic : obesity, myedema, muscle weakness, kyphoscoliosis, upper airway obstruction, diminished respiratory drive, high altitude

Normal or low output. Hypertension due to alveolar hypoxia.

Chronic cor pulmonale

Peripheral edema

Hypoventilation

Cor Pulmonale

Mechanism Responses Characteristic

PULMONARY VASCULAR DISEASE

Emboli, large or multiple Fall in cardiac output due to acute obstruction

Acute cor pulmonale, right ventricular distention, shock

Emboli, small ; vasculitis ; widespread lung damage (ARDS)

Pulmonary hypertension due to widespread hypoxia and microvascular obstruction

Subacute cor pulmonale, right ventricular distention, breastlessness and fever

Emboli, medium, and recurrent; Primary pulmonary hypertension; diet or drug vasopathy

Pulmonary hypertension due to vascular obstruction

Low or normal cardiac output

Chronic cor pulmonale

Right heart hypertrophy

Breathlessness

Note : ARDS = Acute respiratory distress syndrome