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Cor Pulmonale Overview of Cor Pulmonale

Management
Author: Ali A Sovari, MD, FACP; Chief Editor: Henry H Ooi, MD, MRCPI more...
Updated: Oct 06, 2014

Introduction to Cor Pulmonale

Cor pulmonale is defined as an alteration in the structure and function of the right
ventricle caused by a primary disorder of the respiratory system. Pulmonary
hypertension is the common link between lung dysfunction and the heart in cor
pulmonale. Right-sided ventricular disease caused by a primary abnormality of the
left side of the heart or congenital heart disease is not considered cor pulmonale, but
cor pulmonale can develop secondary to a wide variety of cardiopulmonary disease
processes. Although cor pulmonale commonly has a chronic and slowly progressive
course, acute onset or worsening cor pulmonale with life-threatening complications
can occur.[1]

Etiology and Pathophysiology of Cor Pulmonale

Cor pulmonale usually presents chronically, but 2 main conditions can cause acute
cor pulmonale: pulmonary embolism (more common) and acute respiratory distress
syndrome (ARDS). The underlying pathophysiology in massive pulmonary embolism
causing cor pulmonale is the sudden increase in pulmonary resistance. In ARDS, 2
factors cause right ventricular (RV) overload: the pathologic features of the
syndrome itself and mechanical ventilation. Mechanical ventilation, especially higher
tidal volume, requires a higher transpulmonary pressure.
In chronic cor pulmonale, RV hypertrophy (RVH) generally predominates. In acute
cor pulmonale, right ventricular dilatation mainly occurs. In the case of ARDS, cor
pulmonale is associated with increased possibility of right-to-left shunt through the
patent foramen ovale and carries a poorer prognosis.[2]

Several different pathophysiologic mechanisms can lead to pulmonary hypertension

and, subsequently, to cor pulmonale. These pathogenetic mechanisms include the
following:

Pulmonary vasoconstriction due to alveolar hypoxia or blood acidemia This

can result in pulmonary hypertension and if the hypertension is severe
enough, it causes cor pulmonale.
Anatomic compromise of the pulmonary vascular bed secondary to
parenchymal or alveolar lung disorders (eg, emphysema, pulmonary
thromboembolism, interstitial lung disease, adult respiratory distress
syndrome, and rheumatoid disorders) These conditions can cause elevated
pulmonary blood pressure. Chronic obstructive pulmonary disorder is the
most common cause of cor pulmonale, and some connective tissue disorders
with pulmonary involvement may result in pulmonary hypertension and cor
pulmonale.
Increased blood viscosity secondary to blood disorders (eg, polycythemia
vera, sickle cell disease, macroglobulinemia)
Increased blood flow in pulmonary vasculature
Idiopathic primary pulmonary hypertension

The result of the above mechanisms is increased pulmonary arterial pressure.

RV and LV output

The RV is a thin-walled chamber that is more a volume pump than a pressure pump.
It adapts better to changing preloads than afterloads. With an increase in afterload,
the RV increases systolic pressure to keep the gradient. At a point, a further
increase in the degree of pulmonary arterial pressure produces significant RV
dilatation, an increase in RV end-diastolic pressure, and RV circulatory collapse.

A decrease in RV output with a decrease in diastolic left ventricle (LV) volume

results in decreased LV output. Because the right coronary artery, which supplies the
RV free wall, originates from the aorta, decreased LV output diminishes blood
pressure in the aorta and decreases right coronary blood flow. What ensues is a
vicious cycle between decreases in LV and RV output.

RV and LV morphogenesis

Genetic investigations have confirmed that morphogenesis of the right and left
ventricle originated from different sets of progenitor cells and sites. This
polymorphism could explain the differing rates of hypertrophy of the right and left

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ventricles.[3]

RV overload
Right ventricular overload is associated with septal displacement toward the left
ventricle. Septal displacement, which is seen on echocardiography, can be another
factor that decreases LV volume and output in the setting of cor pulmonale and RV
enlargement.

Epidemiology of Cor Pulmonale

Although the prevalence of COPD in the United States is about 15 million, the exact
prevalence of cor pulmonale is difficult to determine, because it does not occur in all
cases of COPD, and the physical examination and routine tests are relatively
insensitive for the detection of pulmonary hypertension.

Cor pulmonale is estimated to account for 6-7% of all types of adult heart disease in
the United States, with chronic obstructive pulmonary disease (COPD) due to
chronic bronchitis or emphysema the causative factor in more than 50% of cases. In
addition, cor pulmonale accounts for 10-30% of decompensated heart failure
related admissions in the United States.[4]
In contrast, acute cor pulmonale is usually secondary to massive pulmonary
embolism. Acute massive pulmonary thromboembolism is the most common cause
of acute life-threatening cor pulmonale in adults; 50,000 deaths in the United States
are estimated to occur per year from pulmonary emboli and about half occur within
the first hour due to acute right heart failure.

Globally, the incidence of cor pulmonale varies among different countries, depending
on the prevalence of cigarette smoking, air pollution, and other risk factors for
various lung diseases.

Cor Pulmonale Presentation

The clinical manifestations of cor pulmonale are generally nonspecific. The

symptoms may be subtle, especially in early stages of the disease, and they may be
mistakenly attributed to the underlying pulmonary pathology.

Symptoms

The patient may complain of fatigue, tachypnea, exertional dyspnea, and cough.
Anginal chest pain can also occur and may be due to right ventricular ischemia (it
usually does not respond to nitrates) or pulmonary artery stretching. A variety of
neurologic symptoms may be seen due to decreased cardiac output and hypoxemia.
Hemoptysis may occur because of rupture of a dilated or atherosclerotic pulmonary
artery. Other conditions, such as tumors, bronchiectasis, and pulmonary infarction,
should be excluded before attributing hemoptysis to pulmonary hypertension.
Rarely, the patient may complain of hoarseness due to compression of the left
recurrent laryngeal nerve by a dilated pulmonary artery.

In advanced stages, passive hepatic congestion secondary to severe right

ventricular failure may lead to anorexia, right upper quadrant abdominal discomfort,
and jaundice. In addition, syncope with exertion, which may also be seen in severe
disease, reflects a relative inability to increase cardiac output during exercise with a
subsequent drop in the systemic arterial pressure.
Elevated pulmonary artery pressure can lead to elevated right atrial, peripheral
venous, and capillary pressure. By increasing the hydrostatic gradient, it leads to
transudation of fluid and accumulation of peripheral edema. Although this is the
simplest explanation for peripheral edema in cor pulmonale, other hypotheses
explain this symptom, especially in a fraction of patients with chronic obstructive
pulmonary disease (COPD) who do not show increase in right atrial pressure. A
decrease in glomerular filtration rate (GFR) and filtration of sodium and stimulation
of arginine vasopressin (which decreases free water excretion) due to hypoxemia
play important pathophysiologic roles in this setting and may even have a role for
peripheral edema in patients with cor pulmonale who have elevated right atrial
pressure.[5]

Signs

Physical findings may reflect the underlying lung disease or pulmonary

hypertension, right ventricular hypertrophy (RVH), and RV failure. An increase in
chest diameter, labored respiratory efforts with retractions of the chest wall,
distended neck veins with prominent a or v waves, and cyanosis may be seen.

On auscultation of the lungs, wheezes and crackles may be heard as signs of

underlying lung disease. Turbulent flow through recanalized vessels in chronic
thromboembolic pulmonary hypertension[6] may be heard as systolic bruits in the
lungs.

Splitting of the second heart sound with accentuation of the pulmonic component

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can be heard in early stages. A systolic ejection murmur with sharp ejection click
over the region of the pulmonary artery may be heard in advanced disease, along
with a diastolic pulmonary regurgitation murmur. Other findings upon auscultation of
the cardiovascular system may be third and fourth sounds of the heart and systolic
murmur of tricuspid regurgitation.

RVH is characterized by a left parasternal or subxiphoid heave. Hepatojugular reflux

and pulsatile liver are signs of RV failure with systemic venous congestion.
On percussion, hyperresonance of the lungs may be a sign of underlying COPD;
ascites can be seen in severe disease.

Examination of the lower extremities reveals evidence of pitting edema. Edema in

cor pulmonale is strongly associated with hypercapnia.[7]

Diagnostic Considerations

A general approach to diagnose cor pulmonale and to investigate its etiology starts
with routine laboratory tests, chest radiography, and electrocardiography.
Echocardiography gives valuable information about the disease and its etiology.
Right heart catheterization is the most accurate but invasive test to confirm the
diagnosis of cor pulmonale and gives important information regarding the underlying
diseases.[8, 9]

Making a diagnosis of cor pulmonale should be followed by further investigation to

determine the underlying lung pathology. Sometimes a common lung disease such
as chronic obstructive pulmonary disease (COPD) is not the only lung pathology as
the cause of cor pulmonale; other lung diseases may coexist. Thus, pulmonary
function tests may become necessary to confirm the underlying lung disease.
Ventilation/perfusion (V/Q) scanning or chest computed tomography (CT) scanning
may be performed if the patients history and physical examination suggest
pulmonary thromboembolism as the cause or if other diagnostic tests do not suggest
other etiologies.
Any abnormal result in each of the above tests may need further diagnostic
evaluation in that specific direction.

Imaging studies may show evidence of underlying cardiopulmonary diseases,

pulmonary hypertension, or its consequence, right ventricular enlargement.

Note the importance of continuous supplemental oxygen therapy in appropriate

patients, as well as the dangers of cigarette smoking while using supplemental
oxygen. Elevation of carboxyhemoglobin in the blood due to smoking can
significantly decrease the effect of O2 on arterial O2 content.

Differentials
When diagnosing cor pulmonale, it is important to consider the possibility of
thromboembolic disease and primary pulmonary hypertension as possible etiologies.
In addition, also assess for the following conditions:
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-sided heart failure due to congenital heart diseases
Ventricular septal defect

Diagnostic Tests

Laboratory investigations are directed toward defining the potential underlying

etiologies as well as evaluating complications of cor pulmonale. In specific
instances, appropriate laboratory studies may include the following:

Hematocrit for polycythemia, which can be a consequence of underlying lung

disease but which can also increase pulmonary arterial pressure by
increasing viscosity
Serum alpha1-antitrypsin, if deficiency is suspected
Antinuclear antibody level for collagen vascular disease, such as
scleroderma
Coagulations studies to evaluate hypercoagulability states (eg, serum levels
of proteins S and C, antithrombin III, factor V Leyden, anticardiolipin
antibodies, homocysteine)

Arterial Blood Gas Analysis

Arterial blood gas measurements may provide important information about the level
of oxygenation and type of acid-base disorder.

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Brain Natriuretic Peptide

Elevated brain natriuretic peptide (BNP) level alone is not adequate to establish the
presence of cor pulmonale, but it helps to diagnose cor pulmonale in conjunction
with other noninvasive tests and in appropriate clinical settings. An elevated BNP
level may actually be a natural mechanism to compensate for elevated pulmonary
hypertension and right heart failure by promoting diuresis and natriuresis,
vasodilating systemic and pulmonary vessels, and reducing circulating levels of
endothelin and aldosterone.

Chest Radiography

In patients with chronic cor pulmonale, the chest radiograph may show enlargement
of the central pulmonary arteries with oligemic peripheral lung fields. Pulmonary
hypertension should be suspected when the right descending pulmonary artery is
larger than 16 mm in diameter and the left pulmonary artery is larger than 18 mm in
diameter. Right ventricular enlargement leads to an increase of the transverse
diameter of the heart shadow to the right on the posteroanterior view and filling of
the retrosternal air space on the lateral view. These findings have reduced sensitivity
in the presence of kyphoscoliosis or hyperinflated lungs.

Electrocardiography

Electrocardiographic (ECG) abnormalities in cor pulmonale reflect the presence of

right ventricular hypertrophy (RVH), RV strain, or underlying pulmonary disease (see
the image below). Such ECG changes may include the following:
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

Severe RVH may reflect as Q waves in the precordial leads that may be mistakenly
interpreted as an anterior myocardial infarction (however, as electrical activity of the
RV is significantly less than the left ventricle [LV], small changes in RV forces may
be lost in the ECG). See the image below.

This ECG shows some typical abnormalities that may be seen in cor pulmonale and other
chronic pulmonary diseases: (1) R/S ratio >1 in V1 and < 1 in V6 suggestive of right
ventricular hypertrophy/enlargement, (2) right superior axis deviation, (3) left atrial type of p
wave with increased width of the p wave and biphasic p wave in V1, and (4) right bundle
branch block pattern with wide QRS and RsR1 pattern in V1 and slurred s wave in V6.This
ECG also presents a sinus bradycardia rhythm with first-degree AV block and left anterior
fascicular block.

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. These
dysrhythmias may be triggered by processes secondary to the underlying disease,
(eg, anxiety, hypoxemia, acid-base imbalance, electrolyte disturbances, excessive
use of bronchodilators, heightened sympathetic activity). Life-threatening ventricular
tachyarrhythmias are less common.
In selected cases, pulmonary function testing may be indicated to determine
underlying obstructive or interstitial lung disease.

2-D and Doppler Echocardiography

Two-dimensional (2-D) echocardiography usually demonstrates signs of chronic

right ventricular (RV) pressure overload. As this overload progresses, 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.
Doppler echocardiography is used to estimate pulmonary arterial pressure, taking

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advantage of the functional tricuspid insufficiency that is usually present in

pulmonary hypertension. This imaging modality is considered the most reliable
noninvasive technique to estimate pulmonary artery pressure. However, the efficacy
of Doppler echocardiography may be limited by the ability to identify an adequate
tricuspid regurgitant jet, which may be further enhanced by using saline contrast.[10]

Pulmonary Thromboembolism Imaging Studies

Pulmonary thromboembolism has a wide range of clinical presentationsfrom

massive embolism with acute and severe hemodynamic instability to multiple
chronic peripheral embolismsthat may present with cor pulmonale.[11]

Ventilation/perfusion (V/Q) lung scanning, pulmonary angiography, and chest

computed tomography (CT) scanning may be indicated to diagnose pulmonary
thromboembolism as the underlying etiology of cor pulmonale. These studies may
be performed early in the diagnostic workup if any evidence of pulmonary embolism
appears in the patients history and physical examination. These evaluations may
also be considered later in the workup if other tests are not suggestive of any other
etiology.[12]

Ultrafast, ECG-gated CT scanning

Ultrafast, electrocardiographically (ECG)-gated computed tomography (CT)

scanning has been evaluated to study right ventricular (RV) function. In addition to
estimating RV ejection fraction (RVEF), this imaging modality can estimate RV wall
mass. Although the use of ultrafast, ECG-gated CT scanning is still experimental,
with further improvement, it may be used to evaluate the progression of cor
pulmonale in the near future.

Magnetic Resonance Imaging

Magnetic resonance imaging of the heart is another modality that can provide
valuable information about right ventricular mass, septal flattening, and ventricular
function.[13, 14]

Nuclear Imaging

Radionuclide ventriculography can noninvasively determine right ventricular ejection

fraction. Myocardial perfusion may also show a permanent increase in brightness of
the right ventricle.[15]

Cardiac Catheterization

Although high-resolution echocardiography and magnetic resonance imaging are

accurate methods to measure pulmonary pressure,[16] right heart catheterization is
considered the most precise method for diagnosis and quantification of pulmonary
hypertension. This procedure is indicated when echocardiography cannot assess
the severity of a tricuspid regurgitant jet, thus excluding an assessment of
pulmonary hypertension.

Right heart catheterization is occasionally important for differentiating cor pulmonale

from occult left ventricular dysfunction, especially when the presentation is
confusing. Another indication is for evaluation of the potential reversibility of
pulmonary arterial hypertension with vasodilator therapy or when a left-sided heart
catheterization is indicated.

Lung Biopsy

Lung biopsy may occasionally be indicated to determine the etiology of underlying

lung disease.

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.[17] However, the approach might be different to some degree in an
acute setting, with priority given to stabilizing the patient.

Cardiopulmonary support for patients experiencing acute cor pulmonale with

resultant acute RV failure includes fluid loading and vasoconstrictor (eg,
epinephrine) administration to maintain adequate blood pressure. Of course, the
primary problem should be corrected, if possible. For example, for massive
pulmonary embolism, consider administration of anticoagulation, thrombolytic agents
or surgical embolectomy, especially if circulatory collapse is impending; consider
bronchodilation and infection treatment in patients with chronic obstructive
pulmonary disease (COPD); and consider steroid and immunosuppressive agents in
infiltrative and fibrotic lung diseases.

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Oxygen therapy, diuretics, vasodilators, digitalis, theophylline, and anticoagulation

therapy are all different modalities used in the long-term management of chronic cor
pulmonale.

Patient education

Patient education regarding the importance of adherence to medical therapy is vital,

because appropriate treatment of both hypoxia and underlying medical illness can
improve mortality and morbidity.

Complications

Complications of cor pulmonale include syncope, hypoxia, pedal edema, passive

hepatic congestion, and death.

Oxygen Therapy

Oxygen therapy is of great importance in patients with underlying chronic obstructive

pulmonary disease (COPD),[18] particularly when administered on a continuous
basis. With cor pulmonale, the partial pressure of oxygen (PaO2) is likely to be below
55 mm Hg and decreases further with exercise and during sleep.
Oxygen therapy relieves hypoxemic pulmonary vasoconstriction, which then
improves cardiac output, lessens sympathetic vasoconstriction, alleviates tissue
hypoxemia, and improves renal perfusion. The multicenter, randomized Nocturnal
Oxygen Therapy Trial (NOTT) showed that continuous low-flow oxygen therapy for
patients with severe COPD resulted in significant reduction in the mortality rate.[19]

In general, in patients with COPD, long-term oxygen therapy is recommended when

the PaO2 is less than 55 mm Hg or the O2 saturation is less than 88%. However, in
the presence of cor pulmonale or impaired mental or cognitive function, long-term
oxygen therapy can be considered even if the PaO2 is greater than 55 mm Hg or the
O2 saturation is greater than 88%.
Although whether oxygen therapy improves survival in patients with cor pulmonale
due to pulmonary disorders other than COPD is not clear, it may provide some
degree of symptomatic relief and improvement in functional status. Therefore,
oxygen therapy plays an important role in both the immediate setting and long-term
management, especially in patients who are hypoxic and have COPD.

Pharmacotherapy

Diuretics are used to decrease the elevated right ventricular (RV) filling volume in
patients with chronic cor pulmonale. Calcium channel blockers are pulmonary artery
vasodilators that have proven efficacy in the long-term management of chronic cor
pulmonale secondary to primary pulmonary arterial hypertension (PAH).[20]

US Food and Drug Administration (FDA)approved prostacyclin analogues and

endothelin-receptor antagonists are available for treatment of primary pulmonary
hypertension (PPH). The beneficial role of cardiac glycosides, namely digitalis, on
the failing right ventricle are somewhat controversial; these agents can improve RV
function but must be used with caution and should be avoided during acute episodes
of hypoxia.
The main indication for oral anticoagulants in the management of cor pulmonale is in
the setting of an underlying thromboembolic event or primary PAH.
Methylxanthines, like theophylline, can be used as an adjunctive treatment for
chronic cor pulmonale secondary to chronic obstructive pulmonary disease (COPD).
Besides the moderate bronchodilatory effect of methylxanthine, this agent improves
myocardial contractility, causes a mild pulmonary vasodilatory effect, and enhances
diaphragmatic contractility.

Diuretic agents

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; however, diuretics may produce hemodynamic adverse
effects if they are not used cautiously. Excessive volume depletion can lead to a
decline in cardiac output.

Another potential complication of diuresis is the production of a hypokalemic

metabolic alkalosis, which diminishes the effectiveness of carbon dioxide stimulation
on the respiratory centers and lessens ventilatory drive. The adverse electrolyte and
acid-base effect of diuretic use can also lead to cardiac arrhythmia, which can
diminish cardiac output. Therefore, diuresis, while recommended in the
management of chronic cor pulmonale, needs to be used with great caution.

Vasodilator drugs

Vasodilators have been advocated in the long-term management of chronic cor

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pulmonale with modest results. Calcium channel blockers, particularly oral

sustained-release nifedipine[21] and diltiazem, can lower pulmonary pressures,
although these agents appear more effective in primary rather than secondary
pulmonary hypertension.[22]

Other classes of vasodilators, such as beta agonists, nitrates, and angiotensinconverting enzyme (ACE) inhibitors have been tried but, in general, vasodilators
have failed to show sustained benefit in patients with COPD, and they are not
routinely used. A trial of vasodilator therapy may be considered only in patients with
COPD with disproportionately high pulmonary blood pressure.

Beta-selective agonist drugs

Beta-selective agonists have an additional advantage of bronchodilator and

mucociliary clearance effect. Right heart catheterization has been recommended
during initial administration of vasodilators to objectively assess the efficacy and
detect the possible adverse hemodynamic consequences of vasodilators.

The FDA approved epoprostenol, treprostinil, bosentan, and iloprost for the
treatment of PPH. Epoprostenol, treprostinil, and iloprost are prostacyclin (PGI2)
analogues and have potent vasodilatory properties.[23] Epoprostenol and treprostinil
are administered intravenously (IV) and iloprost is an inhaler. Bosentan is a mixed
endothelin-A and endothelin-B receptor antagonist indicated for PAH, including PPH.
In clinical trials, bosentan improved exercise capacity, decreased rate of clinical
deterioration, and improved hemodynamics.[23]
The PDE5 inhibitor sildenafil has been intensively studied[24, 25, 26] and was
approved by the FDA for treatment of pulmonary hypertension. Sildenafil promotes
selective smooth muscle relaxation in lung vasculature.[27] Tadalafil, another PDE5
inhibitor, was also approved by the FDA for the treatment of PAH to improve
exercise ability.[28]
There are not enough data available yet regarding the efficacy of these drugs in
patients with secondary pulmonary hypertension, such as in patients with COPD.

Cardiac glycoside agents

The use of cardiac glycosides, such as digitalis, in patients with cor pulmonale has
been controversial, and the beneficial effect of these drugs is not as obvious as in
the setting of left heart failure. Nevertheless, studies have confirmed a modest effect
of digitalis on the failing right ventricle in patients with chronic cor pulmonale.[29] This
drug must be used cautiously, however, and should not be used during the acute
phases of respiratory insufficiency when large fluctuations in levels of hypoxia and
acidosis may occur. Patients with hypoxemia or acidosis are at increased risk of
developing arrhythmias due to digitalis through different mechanisms, including
sympathoadrenal stimulation.

Theophylline

In addition to bronchodilatory effects, theophylline has been reported to reduce

pulmonary vascular resistance and pulmonary arterial pressures acutely in patients
with chronic cor pulmonale secondary to COPD.[30] Theophylline has a weak
inotropic effect and thus may improve right and left ventricular ejection. Low doses
of theophylline have also been suggested to have anti-inflammatory effects that help
to control underlying lung diseases such as COPD.[31] As a result, considering the
use of theophylline as adjunctive therapy in the management of chronic or
decompensated cor pulmonale is reasonable in patients with underlying COPD.

Warfarin

Anticoagulation with warfarin is recommended in patients at high risk for

thromboembolism. The beneficial role of anticoagulation in improving the symptoms
and mortality in patients with primary PAH has been demonstrated in several
studies.[32, 33, 34] The evidence of benefit, however, has not been established in
patients with secondary PAH. Therefore, anticoagulation therapy may be used in
patients with cor pulmonale secondary to thromboembolic phenomena and with
underlying primary PAH.

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. Phlebotomy
results in a decrease in mean pulmonary artery pressure, a decrease in mean
pulmonary vascular resistance,[35] and an improvement in exercise performance in
such patients. However, no evidence suggests improvement in survival.

Generally, phlebotomy should be reserved as an adjunctive therapy for patients with

acute decompensation of cor pulmonale and patients who remain significantly
polycythemic despite appropriate long-term oxygen therapy. Replacement of the
acute volume loss with a saline infusion may be necessary to avoid important
decreases in systemic blood pressure.

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No surgical treatment exists for most diseases that cause chronic cor pulmonale.
Pulmonary embolectomy is efficacious for unresolved pulmonary emboli, which
contribute to pulmonary hypertension. Uvulopalatopharyngoplasty (UPPP) in
selected patients with sleep apnea and hypoventilation may relieve cor
pulmonale.[36]

Single-lung, double-lung, and heart-lung transplantation are all used to salvage the
terminal phases of several diseases (eg, PPH, emphysema, idiopathic pulmonary
fibrosis, cystic fibrosis) complicated by cor pulmonale. Lung transplantation may
lead to a reversal of right ventricular dysfunction from the chronic stress of
pulmonary hypertension. However, strict selection criteria for lung transplant
recipients must be met because of the limited availability of organ donors.

Outpatient Monitoring

Patients with cor pulmonale generally require close attention in the outpatient
setting. It is appropriate to regularly assess the patients oxygen needs and
pulmonary function. Consider a formal program of pulmonary rehabilitation, as many
patients benefit from this therapy.

Prognosis of Cor Pulmonale

The prognosis of cor pulmonale is variable depending upon the underlying

pathology. Development of cor pulmonale as a result of a primary pulmonary
disease usually heralds a poorer prognosis. For example, patients with chronic
obstructive pulmonary disease (COPD) who develop cor pulmonale have a 30%
chance of surviving 5 years. However, whether cor pulmonale carries an
independent prognostic value or is simply reflecting the severity of underlying COPD
or other pulmonary disease is not clear.
Prognosis in the acute setting due to massive pulmonary embolism or acute
respiratory distress syndrome (ARDS) has not previously been shown to be
dependent on the presence or absence of cor pulmonale. However, a prospective,
multicenter cohort study by Volschan et al indicated that in cases of pulmonary
embolism, cor pulmonale may be a predictor of inhospital mortality.[37] The authors
collected demographic, comorbidity, and clinical manifestation data on 582 patients
admitted to emergency or intensive care units and diagnosed with pulmonary
embolism. Assessing the information using logistic regression analysis, the
investigators built a prediction model. Their results indicated that in
hemodynamically stable patients with pulmonary embolism, the following factors
may be independent predictors of inhospital mortality[37] :
Age older than 65 years
Bed rest for longer than 72 hours
Chronic cor pulmonale
Sinus tachycardia
Tachypnea

A Chinese study indicated that chronic cor pulmonale is one of the major risk factors
for early hospital readmission in patients following hospitalization for acute
exacerbation of COPD. The study, by Lin et al, of 692 patients, included 63 patients
who were readmitted to the hospital within 31 days after discharge. Through
multivariate analysis, the investigators found that risk factors for early readmission
included, in order of significance, chronic cor pulmonale (odds ratio [OR], 2.14),
hypoproteinemia (OR, 2.02), and an elevated partial pressure of CO2 (Pa CO2 [OR,
1.03]).[38]

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Contributor Information and Disclosures

Author
Ali A Sovari, MD, FACP Clinical and Research Fellow in Cardiovascular Medicine, Section of Cardiology,
University of Illinois College of Medicine; Staff Physician and Hospitalist, St John Regional Medical Center,
Cogent Healthcare, Inc
Ali A Sovari, MD, FACP is a member of the following medical societies: American College of Cardiology, American
College of Physicians, American Heart Association, American Medical Association, American Physiological
Society, Heart Rhythm Society
Disclosure: Nothing to disclose.

Coauthor(s)
Abraham G Kocheril, MD, FACC, FACP, FHRS Professor of Medicine, University of Illinois College of Medicine
Abraham G Kocheril, MD, FACC, FACP, FHRS is a member of the following medical societies: American College
of Cardiology, Central Society for Clinical and Translational Research, Heart Failure Society of America, Cardiac
Electrophysiology Society, American College of Physicians, American Heart Association, American Medical
Association, Illinois State Medical Society
Disclosure: Nothing to disclose.

Ravi H Dave, MD Associate Professor of Medicine, University of California at Los Angeles David Geffen School
of Medicine
Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College
of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.

Steven J Compton, MD, FACC, FACP, FHRS Director of Cardiac Electrophysiology, Alaska Heart Institute,
Providence and Alaska Regional Hospitals
Steven J Compton, MD, FACC, FACP, FHRS is a member of the following medical societies: American College of
Physicians, American Heart Association, American Medical Association, Heart Rhythm Society, Alaska State
Medical Association, American College of Cardiology
Disclosure: Nothing to disclose.

Chief Editor
Henry H Ooi, MD, MRCPI Director, Advanced Heart Failure and Cardiac Transplant Program, Nashville Veterans
Affairs Medical Center; Assistant Professor of Medicine, Vanderbilt University School of Medicine
Disclosure: Nothing to disclose.

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