COR PULMONALE

Gusti Ayu Suryawati

Definition
Cor Pulmonale
pulmonary heart disease
dilation and hypertrophy of the right ventricle
(RV) in response to diseases of the pulmonary
vasculature and/or lung parenchyma.

excluded congenital heart disease and those

diseases in which the right heart fails
secondary to dysfunction of the left side of the
heart
Etiology and
Epidemiology
develops in response to acute or chronic
changes in the pulmonary vasculature
Changes that are sufficient to cause
pulmonary hypertension

Once patients with chronic pulmonary or

pulmonary vascular disease develop cor
pulmonale, their prognosis worsens
Pathophysiology
pulmonary hypertension that is sufficient to
lead to RV dilation, with or without the
development of concomitant RV
hypertrophy
Right ventricle: thin walled, compliant
Better suited for high volumes than high
pressure
Sustained pressure overload (pulm HPN)
and increased vascular resistance causes
RV to fail
Pathophysiology
Acute Cor Pulmonale
occurs after a sudden and severe stimulus with
RV dilatation and failure but no RV hypertrophy
e.g massive pulmonary embolus
Chronic Cor pulmonale
more slowly evolving and slowly progressive
pulmonary hypertension that leads to RV
dilation and hypertrophy
 Acute cor pulmonale: pulmonary embolism
(more common) and acute respiratory
distress syndrome (ARDS).
The underlying pathophysiology in a
massive pulmonary embolism causing cor
pulmonale is the sudden increase in
pulmonary resistance.
In ARDS, RV overload can occur due to
mechanical ventilation and the pathologic
features of the syndrome itself.
 The World Health Organization (WHO) has
five classifications for pulmonary
hypertension, and all except one of these
groups can result in cor pulmonale (WHO
Classification group 2 is pulmonary artery
hypertension due to left ventricular [LV]
dysfunction). Note the following WHO
classifications:
 Group 1: Pulmonary artery
hypertension, including heritable
causes; connective-tissue disorders,
including scleroderma; and other
idiopathic causes
Group 3: Pulmonary hypertension due
to lung disease and/or hypoxia; these
disorders include chronic obstructive
pulmonary disease (COPD), which is the
most common cause of for pulmonale.
 Group 4: Chronic thromboembolic
pulmonary hypertension; blood clots that
form in the lungs can lead to increased
resistance, pulmonary hypertension and,
subsequently, cor pulmonale
Group 5: Pulmonary hypertension caused
by other diseases or conditions, including
sarcoidosis, polycythemia vera (which can
lead to increased blood viscosity and,
subsequently, pulmonary hypertension),
vasculitis, and other disorders.
Pathophysiology
Factors that determine
severity
hypoxia secondary to alterations in gas
exchange
Hypercapnia
Acidosis
alterations in RV volume overload that are
affected by:
exercise, heart rate, polycythemia, or increased salt
and retention because of a fall in cardiac output
Clinical presentation
Symptoms:
Dyspnea, the most common symptom
usually the result of the increased work of breathing
secondary to changes in elastic recoil of the lung (fibrosing
lung diseases) or altered respiratory mechanics
Orthopnea and paroxysmal nocturnal dyspnea
are rarely symptoms of isolated right HF
reflect the increased work of breathing in the supine
position that results from compromised excursion of the
diaphragm
Clinical presentation
Symptoms:
effort-related syncope
because of the inability of the RV to deliver blood
adequately to the left side of the heart
Abdominal pain,anorexia and ascites
Due to right heart failure
Lower extremity edema
secondary to neurohormonal activation, elevated RV filling
pressures, or increased levels of carbon dioxide and
hypoxia,
 Hemoptoe
due to rupture of a dilated or atherosclerotic
pulmonary arteriole
Anginal chest pain can also occur and may be due
to right ventricular ischemia or pulmonary artery
stretching
hoarseness due to compression of the left
recurrent laryngeal nerve by a dilated pulmonary
artery
Clinical presentation

Signs
tachypnea
elevated jugular venous pressures
hepatomegaly
lower-extremity edema
Cyanosis is a late finding
wheezes and crackles
hyperresonance of the lungs
Splitting of the second heart sound
Sign:
A systolic ejection murmur with a
sharp ejection click over the region of
the pulmonary artery
a diastolic pulmonary regurgitation
murmur
systolic murmur of tricuspid
regurgitation.
Sign
left parasternal or subxiphoid heave.
Hepatojugular reflux and pulsatile liver are
signs of RV failure with systemic venous
congestion
Diagnosis

ECG
P pulmonale, right axis deviation, and RV
hypertrophy
Chest X Ray
enlargement of the main pulmonary
artery, hilar vessels, and the descending
right pulmonary artery
CT,MRI
Differential Dx/

Atrial myxoma
Blood disorders that are associated with increased
blood viscosity
Congestive (biventricular) heart failure
Constrictive pericarditis
High-output heart failure
Infiltrative cardiomyopathies
Primary pulmonic stenosis
Right heart failure due to right ventricular infarction
Right heart failure due to congenital heart diseases
Ventricular septal defect
Laboratorium
Hematocrit for polycythemia
Coagulations studies to evaluate
hypercoagulability states
Arterial blood gas
Brain natriuretic peptide (BNP)
Chest Radiography
In patients with chronic cor pulmonale,
the chest radiograph may show
enlargement of the central pulmonary
arteries with oligemic peripheral lung
fields.
ECG
Right axis deviation
R/S amplitude ratio in V1 greater than 1 (an
increase in anteriorly directed forces may be a sign
of posterior infarction)
R/S amplitude ratio in V6 less than 1
P-pulmonale pattern (an increase in P wave
amplitude in leads 2, 3, and aVF)
S 1 Q 3 T 3 pattern and incomplete (or complete)
right bundle branch block, especially if pulmonary
embolism is the underlying etiology
Low-voltage QRS because of underlying COPD
with hyperinflation
 Additionally, many rhythm
disturbances may be present in
chronic cor pulmonale; these range
from isolated premature atrial
depolarizations to various
supraventricular tachycardias,
including paroxysmal atrial
tachycardia, multifocal atrial
tachycardia, atrial fibrillation, atrial
flutter, and junctional tachycardia.
2-D and Doppler Echocardiography
signs of chronic right ventricular (RV)
pressure overload. increased thickness of
the RV wall with paradoxical motion of the
interventricular septum during systole occurs.
At an advanced stage, RV dilatation occurs,
and the septum shows abnormal diastolic
flattening.
In extreme cases, the septum may actually
bulge into the left ventricular (LV) cavity
during diastole, resulting in decreased LV
diastolic volume and reduction of LV output.
 tricuspid insufficiency
tricuspid annular plane systolic
excursion (TAPSE) for global RV
function.
Pulmonary Thromboembolism
Imaging Studies
Pulmonary angiography
computed tomography pulmonary
angiography (CTPA)
CMR. Such a technique can be useful
in determining the size and location of
an infarction.
Nuclear imaging
 Right heart catheterization
Lung biopsy
Overview of Cor Pulmonale
Management

Medical therapy for chronic cor pulmonale

is generally focused on treatment of the
underlying pulmonary disease and
improving oxygenation and right ventricular
(RV) function by increasing RV contractility
and decreasing pulmonary
vasoconstriction.
Management
acute RV failure includes fluid loading and
vasoconstrictor
anticoagulation, thrombolytic agents or
surgical
bronchodilation and infection treatment in
patients with chronic obstructive pulmonary
disease (COPD)
consider steroid and immunosuppressive
agents in infiltrative and fibrotic lung
diseases.
Management
Oxygen therapy
relieves hypoxemic pulmonary
vasoconstriction, improves cardiac
output, lessens sympathetic
vasoconstriction, alleviates tissue
hypoxemia, and improves renal
perfusion.
Pharmacotherapy

Calcium channel blockers are pulmonary

artery vasodilators that have some efficacy
in the long-term management of chronic cor
pulmonale secondary to primary pulmonary
arterial hypertension (PAH).
 Diuretics are used in the management of
chronic cor pulmonale, particularly when
the RV filling volume is markedly elevated
and in the management of associated
peripheral edema. These agents may result
in improvement of the function of both the
right and left ventricles
 prostacyclin analogues and endothelin-
receptor antagonists are available for
treatment of pulmonary arterial
hypertension (PAH).
 moderate bronchodilatory effect of
methylxanthine, this agent improves
myocardial contractility, causes a mild
pulmonary vasodilatory effect, and
enhances diaphragmatic contractility
 Vasodilators have been advocated in the
long-term management of chronic cor
pulmonale with modest results.
Calcium channel blockers, particularly oral
sustained-release nifedipine [24] and
diltiazem, can lower pulmonary pressures,
although these agents appear more
effective in primary rather than secondary
pulmonary hypertension. [25]
 Beta-selective agonists have an additional
advantage of bronchodilator and
mucociliary clearance effect.
theophylline has been reported to reduce
pulmonary vascular resistance and
pulmonary arterial pressures acutely in
patients with chronic cor pulmonale
secondary to COPD.
 Anticoagulation with warfarin is
recommended in patients at high risk for
thromboembolism.
Thrombolytic therapy is indicated in
patients with acute cor pulmonale due to a
pulmonary embolism resulting in
hemodynamic instability
Surgical Management of
Cor Pulmonale
Phlebotomy is indicated in patients with
chronic cor pulmonale and chronic hypoxia
causing severe polycythemia, defined as
hematocrit of 65% or more.
Pulmonary embolectomy
Single-lung, double-lung, and heart-lung
transplantation are all used to salvage the
terminal phases of several diseases
Treatment
Primary goal: target the underlying
pulmonary disease
decrease in pulmonary vascular resistance and
relieve the pressure overload on the RV
General principles:
decreasing the work of breathing using noninvasive
mechanical ventilation, bronchodilation, and steroids
treating any underlying infection
Adequate oxygenation (oxygen saturation 90
92%) will also decrease pulmonary vascular
resistance and reduce the demands on the RV
Diuretics
 The pathophysiology of cor pulmonale is a
result of increased right-sided filling
pressures from pulmonary hypertension
that is associated with diseases of the lung.
The increased afterload leads to structural
alterations in the right ventricle (RV)
including RV hypertrophy (RVH) which can
be seen in chronic cor pulmonale
Diagnosis
2D echo
measuring RV thickness and chamber
dimensions
Doppler echocardiography
assess pulmonary artery pressures
MRI
assessing RV structure and function,
particularly in patients who are difficult to
image with 2-D echocardiography because of
severe lung disease