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Akhnal Syaputra
Cor Pulmonal Chronic
Definisi
Kor pulmonal kronis adalah hipertroIi dan dilatasi ventrikel kanan akibat hipertensi pulnomal
yang disebabkan oleh penyakit paru kronis. Pada perkembangannya akan berlanjut menjadi gagal
jantung kanan.
Etiologi
Penyakit yang mendasari terjadinya kor pulmonal dapat digolongkan mejadi 4 kelompok :
1. Penyakit pembuluh darah paru.
2. Penekanan pada arteri pulmonal oleh tumor mediastinum, aneurisma, granuloma atau
Iibrosis.
3. Penyakit neuro muskular dan dinding dada.
4. Penyakit yang mengenai aliran udara paru, alveoli, termasuk Penyakit Paru ObstruktiI
Kronis (PPOK), penyakit paru interstisial dan gangguan pernaIasaan saat tidur.
Penyakit yang menjadi penyebab utama dari kor pulmonal kronis adalah PPOK, diperkirakan 80-
90 kasus.
Patofisiologi
Penyakit paru kronis akan mengakibatkan :
1. erkurangnya ';askular bed paru, dapat disebabkan oleh semakin terdesaknya
pembuluh darah oleh paru yang mengembang atau kerusakan paru
2. Asidosis dan hiperkapnia
3. Hipoksia alveolar, yang akan merangsang vasokontriksi pembuluh darah
4. Polisitemia dan hiperviskositas darah.
Keempat kelainan ini akan menyebabkan timbulnya hipertensi pulmonal. Dalam jangka panjang
akan mengakibatkan hipertroIi dan dilatasi ventrikel kanan dan kemudian berlanjut menjadi
gagal jantung kanan.
Diagnosis
Pada anamnesis, biasanya pasien mengeluhkan :
O atigue, takipnue, exertional dyspnea, dan batuk
O yeri dada atau angina yang disebabkan oleh iskemia pada ventrikel kanan atau
teregangnya arteri pulmonalis.
O Hemoptisis, karena rupturnya arteri pulmonalis yang sudah mengalami arteroslerotik atau
terdilatasi akibat hipertensi pulmonal.
O isa juga ditemukan variasi gejala-gejala neurologis, akibat menurunnya curah jantung
dan hipoksemia.
O Pada tahap lanjut, gagal jantung kanan akan mengakibatkan kongestiI hepar, sehingga
muncul gejala anoreksia, nyeri atau rasa tidak nyaman pada perut kanan atas, dan ikterus.
Selanjutnya pada pemeriksaan Iisik, kita bisa mendapatkan :
O nspeksi : diameter dinding dada yang membesar, sianosis
O Palpasi : edema tungkai, peningkatan vena jugularis yang menandakan terjadinya gagal
jantung kanan.
O Perkusi : pada paru bisa terdengar hipersonor pada PPOK, pada keadaan yang berat bisa
menyebabkan asites.
O Auskultasi :pada paru ditemukan hee:ing dan rhonki, bisa juga ditemukan bising
sistolik di paru akibat turbulensi aliran pada rekanalisasi pembuluh darah pada chronic
thromboembolic pulmonary hypertension. Split pada bunyi jantung , dapat ditemukan
pada tahap awal, namun pada tahap lanjut dapat terdengar systolic efection murmur yang
terdengar lebih keras di area pulmonal. unyi jantung dan ' juga terdengar serta
mumur sistolik dari regurgitasi pulmonal.
Pada pemeriksaan penunjang:
O Pada Ioto thorak, ditemukan corakan vaskuler meningkat, pelebaran hilus dan trunkus
pulmolnal. Kemudian tanda-tanda pembesaran ventrikel kanan, seperti apeks terangkat,
pinggang jantung menghilang.
O Pada EKG, ditemukan gelompang P pulmonal, deviasi aksis jantung ke kanan dan R'H.
O Pada EchokardiograIi ditemukan penebalan dinding ventrikel kanan, pelebaran rongga
ventrikel kanan ke arah kiri, septum interventrikuler bergeser ke kiri dan bergerak
berlawanan selama siklus jantung.
O Kateterisasi jantung, akan membantu untuk menilai tekanan vaskuler paru, kalkulasi
tahanan vaskular paru serta responnya terhadap pemberian oksigen dan vasolilator.
Penatalaksanaan
Tujuan dari terapi pada kor pulmonal kronik adalah :
1. Mengoptimalkan eIisiensi pertukaran gas
2. Menurunkan hipertensi pulmonal
3. Meningkatkan kelangsungan hidup
4. Pengobatan penyakit dasar dan komplikasinya.
Penatalaksanaan tentu diawali dengan istirahat, diet jantung yang rendah garam, kemudian
menghentikan Iaktor resiko seperti merokok pada pasien PPOK. Kemudian penatalaksanaan
selanjutnya sebagai berikut :
%erapi Oksigen
Mekanisme bagaimana terapi oksigen dapat menigkatkan kelangsungan hidup belum diketahui
pasti, namun ada 2 hipotesis : (1) terapi oksigen mengurangi vasikontriksi dan menurunkan
resistensi vaskular paru yang kemuadian meningkatkan isi sekuncup ventrikel kanan. (2) terapi
oksigen meningkatkan kadar oksigen arteri dan meningkatkan hantaran oksigen ke jantung, otak
dan organ vital lainnya.
Pemakaian oksigen secara kontinyu selama 12 jam (ational Institute of Health, USA); 15 jam
(British Medical Research Counsil) meningkatkan kelangsungan hidup dibanding pasien tanpa
terapi oksigen. ndikasi terapi oksigen adalah : PaO2 _ 55 mmHg atau SaO2 _ 88, PaO2 55-59
mmHg disertai salah satu dari : edema disebabkan gagal jantung kanan, P pulmonal pada EKG,
eritrositosis hematokrit ~ 56.
'asodilator
Pemakaian vasodilator seperti nitrat, hidralazin, antagonis kalsium, agonis alIa adrenergik, ACE-
, dan postaglandin belum direkomendasikan secara rutin. 'asodilator dapat menurunkan
tekanan pulmonal pada kor pulmonal kronik, meskipun eIisiensinya lebih baik pada hipertensi
pulmonal yang primer. 'asodilator yang biasa dipakai adalah niIedipine dengan dosis 10-30 mg
PO 3 kali sehari, maksimal 120 -180 mg per hari.
Digitalis
Hanya digunakan pada pasien kol pulmonal bisa disertai gagal jantung kiri. Digoksin bisa
diberikan dengan dosis 0,125-0,375 mg PO 1 x 1. Pada pemberian digitalis perlu diwaspadai
resiko aritmia.
Diuretik
Diberikan bila ditemukan gagal jantung kanan, pemberian diuretik berlebihan dapat
menimbulkan alkalosis metabolik yang dapat memicu peningkatan hiperkapnia. Disamping itu
pemberian diuretik dapat menimbulkan kekurangan cairan sehingga mengakibatkan preload
ventrikel kanan dan curah jantung menurun. Furosemid dapat diberikan dengan dosis 20-80 mg
per hari PO / ', dosis maksimal 600 mg per hari.
Antikoagulan
Diberikan untuk menurunkan resiko terjadinya tombroemboli akibat disIungsi dan pembesaran
ventrikel kanan adanya Iaktor imobilisasi pada pasien. WarIarin dapat diberikan dengan dosis 2-
10 mg PO 1 x 1.
Prognosis
Pada kor pulmonal kronik yang disertai gagal jantung kanan, prognosisnya buruk. amun
dengan pemberian terapi oksigen dalam jangka panjang dapat meningkatkan kualitas hidup
pasien.
epustakaan
1. Sjaharuddin Harun dan ka Prasepta Wijaya, Kor Pulmonal Kronik, dalam uku Ajar
lmu Penyakit Dalam Jilid Edisi '. nternaPublishing2009; 287: 1842-44
2. raunwald E, Heart Failure and cor pulmonale, dalam Harisson`s Principles nternal
Medicine, edisi 16. ew York, McGraw-Hill, 2005; 216 : 1367-78
3. Weitzenblum E, Chronic Cor Pulmonale. Heart 2003; 89:225-30. Diakses dari situs
www.bmj-heart.com tanggal 18 desember 2010.
4. Ali A Sovari, Cor Pulmonale. University oI llinois at Chicago, 2010. Diakses dari situs
www.emedicine.com tanggal 18 desember 2010

Cor pulmonale is right ventricular enlargement secondary to a lung disorder that causes
pulmonary artery hypertension. Right ventricular Iailure Iollows. Findings include peripheral
edema, neck vein distention, hepatomegaly, and a parasternal liIt. Diagnosis is clinical and by
echocardiography. Treatment is directed at the cause.
Cor pulmonale results Irom a disorder oI the lung or its vasculature; it does not reIer to right
ventricular (R') enlargement secondary to leIt ventricular (L') Iailure, a congenital heart
disorder (eg, ventricular septal deIect), or an acquired valvular disorder. Cor pulmonale is
usually chronic but may be acute and reversible. Primary pulmonary hypertension (ie, not caused
by a pulmonary or cardiac disorder) is discussed elsewhere

Pathophysiology
Lung disorders cause pulmonary hypertension by several mechanisms:
O Loss oI capillary beds (eg, due to bullous changes in COPD or thrombosis in pulmonary
embolism)
O 'asoconstriction caused by hypoxia, hypercapnia, or both
O ncreased alveolar pressure (eg, in COPD, during mechanical ventilation)
O Medial hypertrophy in arterioles (oIten a response to pulmonary hypertension due to
other mechanisms)
Pulmonary hypertension increases aIterload on the R', resulting in a cascade oI events that is
similar to what occurs in L' Iailure, including elevated end-diastolic and central venous pressure
and ventricular hypertrophy and dilation. Demands on the R' may be intensiIied by increased
blood viscosity due to hypoxia-induced polycythemia. Rarely, R' Iailure aIIects the L' iI a
dysIunctional septum bulges into the L', interIering with Iilling and thus causing diastolic
dysIunction.
Etiology
Acute cor pulmonale has Iew causes. Chronic cor pulmonale is usually caused by COPD, but
there are several less common causes (see Table 3: Heart Failure: Causes oI Cor Pulmonale ).
n patients with COPD, an acute exacerbation or pulmonary inIection may trigger R' overload.
n chronic cor pulmonale, risk oI venous thromboembolism is increased.
Symptoms and Signs
nitially, cor pulmonale is asymptomatic, although patients usually have signiIicant symptoms
due to the underlying lung disorder (eg, dyspnea, exertional Iatigue). Later, as R' pressures
increase, physical signs commonly include a leIt parasternal systolic liIt, a loud pulmonic
component oI the 2nd heart sound (S
2
), and murmurs oI Iunctional tricuspid and pulmonic
insuIIiciency. Later, an R' gallop rhythm (3rd |S
3
| and 4th |S
4
| heart sounds) augmented during
inspiration, distended jugular veins (with a dominant a wave unless tricuspid regurgitation is
present), hepatomegaly, and lower-extremity edema may occur.
Diagnosis
O Clinical suspicion
O Echocardiography
Cor pulmonale should be suspected in all patients with one oI its causes. Chest x-rays show R'
and proximal pulmonary artery enlargement with distal arterial attenuation. ECG evidence oI R'
hypertrophy (eg, right axis deviation, QR wave in lead '
1
, and dominant R wave in leads '
1
to
'
3
) correlates well with degree oI pulmonary hypertension. However, because pulmonary
hyperinIlation and bullae in COPD cause realignment oI the heart, physical examination, x-rays,
and ECG may be relatively insensitive. Echocardiography or radionuclide imaging is done to
evaluate L' and R' Iunction; echocardiography can assess R' systolic pressure but is oIten
technically limited by the lung disorder. Right heart catheterization may be required Ior
conIirmation.
Treatment
O Treatment oI cause
Treatment is diIIicult; it Iocuses on the cause (see elsewhere in The Manual), particularly
alleviation or moderation oI hypoxia. Early identiIication and treatment are important beIore
structural changes become irreversible.
I peripheral edema is present, diuretics may seem appropriate, but they are helpIul only iI L'
Iailure and pulmonary Iluid overload are also present; they may be harmIul because small
decreases in preload oIten worsen cor pulmonale. Pulmonary vasodilators (eg, hydralazineSome
Trade ames
APRESOLE
Click Ior Drug Monograph
, Ca channel blockers, nitrous oxide, prostacyclin, phosphodiesterase inhibitors), although
beneIicial in primary pulmonary hypertension, are not eIIective. osentanSome Trade ames
TRACLEER
Click Ior Drug Monograph
, an endothelin receptor blocker, also may beneIit patients with primary pulmonary hypertension,
but its use is not well studied in cor pulmonale. DigoxinSome Trade ames
DGTEK
LAOX
Click Ior Drug Monograph
is eIIective only iI patients have concomitant L' dysIunction; caution is required because
patients with COPD are sensitive to digoxinSome Trade ames
DGTEK
LAOX
Click Ior Drug Monograph
s eIIects. Phlebotomy during hypoxic cor pulmonale has been suggested, but the beneIits oI
decreasing blood viscosity are not likely to oIIset the harm oI reducing O
2
-carrying capacity
unless signiIicant polycythemia is present. For patients with chronic cor pulmonale, long-term
anticoagulants reduce risk oI venous thromboembolism.

Pharmacotherapy
Diuretics are used to decrease the elevated right ventricular (R') Iilling volume in patients with
chronic cor pulmonale. Calcium channel blockers are pulmonary artery vasodilators that have
proven eIIicacy in the long-term management oI chronic cor pulmonale secondary to primary
pulmonary arterial hypertension (PAH).
|14|

US Food and Drug Administration (FDA)approved prostacyclin analogues and endothelin-
receptor antagonists are available Ior treatment oI primary pulmonary hypertension (PPH). The
beneIicial role oI cardiac glycosides, namely digitalis, on the Iailing right ventricle are somewhat
controversial; these agents can improve R' Iunction but must be used with caution and should
be avoided during acute episodes oI hypoxia.
The main indication Ior oral anticoagulants in the management oI cor pulmonale is in the setting
oI an underlying thromboembolic event or primary PAH.
Methylxanthines, like theophylline, can be used as an adjunctive treatment Ior chronic cor
pulmonale secondary to chronic obstructive pulmonary disease (COPD). esides the moderate
bronchodilatory eIIect oI methylxanthine, this agent improves myocardial contractility, causes a
mild pulmonary vasodilatory eIIect, and enhances diaphragmatic contractility.
Diuretic agents
Diuretics are used in the management oI chronic cor pulmonale, particularly when the R' Iilling
volume is markedly elevated and in the management oI associated peripheral edema. These
agents may result in improvement oI the Iunction oI both the right and leIt ventricles; however,
diuretics may produce hemodynamic adverse eIIects iI they are not used cautiously. Excessive
volume depletion can lead to a decline in cardiac output.
Another potential complication oI diuresis is the production oI a hypokalemic metabolic
alkalosis, which diminishes the eIIectiveness oI carbon dioxide stimulation on the respiratory
centers and lessens ventilatory drive. The adverse electrolyte and acid-base eIIect oI diuretic use
can also lead to cardiac arrhythmia, which can diminish cardiac output. ThereIore, diuresis,
while recommended in the management oI chronic cor pulmonale, needs to be used with great
caution.
'asodilator drugs
'asodilators have been advocated in the long-term management oI chronic cor pulmonale with
modest results. Calcium channel blockers, particularly oral sustained-release niIedipine
|15|
and
diltiazem, can lower pulmonary pressures, although these agents appear more eIIective in
primary rather than secondary pulmonary hypertension.
|16|

Other classes oI vasodilators, such as beta agonists, nitrates, and angiotensin-converting enzyme
(ACE) inhibitors have been tried but, in general, vasodilators have Iailed to show sustained
beneIit in patients with COPD, and they are not routinely used. A trial oI vasodilator therapy
may be considered only in patients with COPD with disproportionately high pulmonary blood
pressure.
eta-selective agonist drugs
eta-selective agonists have an additional advantage oI bronchodilator and mucociliary
clearance eIIect. Right heart catheterization has been recommended during initial administration
oI vasodilators to objectively assess the eIIicacy and detect the possible adverse hemodynamic
consequences oI vasodilators.
The FDA approved epoprostenol, treprostinil, bosentan, and iloprost Ior the treatment oI PPH.
Epoprostenol, treprostinil, and iloprost are prostacyclin (PG2) analogues and have potent
vasodilatory properties.
|17|
Epoprostenol and treprostinil are administered intravenously (') and
iloprost is an inhaler. osentan is a mixed endothelin-A and endothelin- receptor antagonist
indicated Ior PAH, including PPH. n clinical trials, bosentan improved exercise capacity,
decreased rate oI clinical deterioration, and improved hemodynamics.
|17|

The PDE5 inhibitor sildenaIil has been intensively studied
|18, 19, 20|
and was approved by the FDA
Ior treatment oI pulmonary hypertension. SildenaIil promotes selective smooth muscle relaxation
in lung vasculature.
|21|
TadalaIil, another PDE5 inhibitor, was also approved by the FDA Ior the
treatment oI PAH to improve exercise ability.
|22|

There are not enough data available yet regarding the eIIicacy oI these drugs in patients with
secondary pulmonary hypertension, such as in patients with COPD.
Cardiac glycoside agents
The use oI cardiac glycosides, such as digitalis, in patients with cor pulmonale has been
controversial, and the beneIicial eIIect oI these drugs is not as obvious as in the setting oI leIt
heart Iailure. evertheless, studies have conIirmed a modest eIIect oI digitalis on the Iailing right
ventricle in patients with chronic cor pulmonale.
|23|
This drug must be used cautiously, however,
and should not be used during the acute phases oI respiratory insuIIiciency when large
Iluctuations in levels oI hypoxia and acidosis may occur. Patients with hypoxemia or acidosis are
at increased risk oI developing arrhythmias due to digitalis through diIIerent mechanisms,
including sympathoadrenal stimulation.
%heophylline
n addition to bronchodilatory eIIects, theophylline has been reported to reduce pulmonary
vascular resistance and pulmonary arterial pressures acutely in patients with chronic cor
pulmonale secondary to COPD.
|24|
Theophylline has a weak inotropic eIIect and thus may
improve right and leIt ventricular ejection. Low doses oI theophylline have also been suggested
to have anti-inIlammatory eIIects that help to control underlying lung diseases such as COPD.
|25|
As a result, considering the use oI theophylline as adjunctive therapy in the management oI
chronic or decompensated cor pulmonale is reasonable in patients with underlying COPD.
arfarin
Anticoagulation with warIarin is recommended in patients at high risk Ior thromboembolism.
The beneIicial role oI anticoagulation in improving the symptoms and mortality in patients with
primary PAH has been demonstrated in several studies.
|26, 27, 28|
The evidence oI beneIit,
however, has not been established in patients with secondary PAH. ThereIore, anticoagulation
therapy may be used in patients with cor pulmonale secondary to thromboembolic phenomena
and with underlying primary PAH.