9. Cor Pulmonale

Cor Pulmonale Cor Pulmonale

BackgroundBackground

Cor pulmonaleCor pulmonale == alteration in alteration in the structure and function of the the structure and function of the right ventricle caused by a primary right ventricle caused by a primary disorder of the respiratory system.disorder of the respiratory system.

Pulmonary hypertensionPulmonary hypertension is the is the common link between lung dysfunction common link between lung dysfunction and the heart in cor pulmonale. and the heart in cor pulmonale.

Although cor pulmonale commonly has Although cor pulmonale commonly has a chronic and slowly progressive a chronic and slowly progressive course, acute onset or worsening cor course, acute onset or worsening cor pulmonale with life-threatening pulmonale with life-threatening complications can occur.complications can occur.

PathophysiologyPathophysiology

pulmonary vasoconstrictionpulmonary vasoconstriction due to due to alveolar hypoxia or blood acidemiaalveolar hypoxia or blood acidemia

anatomic compromise of the pulmonary anatomic compromise of the pulmonary vascular bed secondaryvascular bed secondary to lung disorders to lung disorders (eg, emphysema, pulmonary (eg, emphysema, pulmonary thromboembolism, interstitial lung disease)thromboembolism, interstitial lung disease)

increased blood viscosityincreased blood viscosity secondary to secondary to blood disorders (eg, polycythemia vera, blood disorders (eg, polycythemia vera, sickle cell disease, macroglobulinemia) sickle cell disease, macroglobulinemia)

idiopathic primary pulmonary idiopathic primary pulmonary hypertensionhypertension..

The result is The result is increased pulmonary arterial increased pulmonary arterial pressurepressure. .


The right ventricleThe right ventricle (RV) is a thin-walled (RV) is a thin-walled chamber that is more a volume pump than a chamber that is more a volume pump than a pressure pumppressure pump;; iit adapts better to changing t adapts better to changing preloads than afterloads.preloads than afterloads.

With an increase in afterload, the With an increase in afterload, the RV RV increases systolic pressure to keep the increases systolic pressure to keep the gradient.gradient.

At a point, a further increase in the degree of At a point, a further increase in the degree of pulmonary arterial pressure produces significant pulmonary arterial pressure produces significant RV dilationRV dilation, an increase in RV end-diastolic , an increase in RV end-diastolic pressure, and RV circulatory collapse. pressure, and RV circulatory collapse.

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


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


Right ventricular overloadRight ventricular overload is associated is associated with with septal displacement toward the left septal displacement toward the left ventricleventricle..

Septal displacementSeptal displacement, which is seen on , which is seen on echocardiography, can be another factor that echocardiography, can be another factor that decreases LV volume and outputdecreases LV volume and output in the in the setting of cor pulmonale and right ventricular setting of cor pulmonale and right ventricular enlargement. enlargement.

Chronic obstructive pulmonary diseaseChronic obstructive pulmonary disease (COPD) is the most common cause of cor (COPD) is the most common cause of cor pulmonale in the United States. pulmonale in the United States.


Cor pulmonale usually presents chronically, but Cor pulmonale usually presents chronically, but 2 main conditions can cause 2 main conditions can cause acute cor acute cor pulmonalepulmonale::

massive massive pulmonary embolismpulmonary embolism (more (more common) common)

acute respiratory distress syndromeacute respiratory distress syndrome (ARDS). (ARDS).

The underlying pathophysiology in The underlying pathophysiology in massive massive pulmonary embolismpulmonary embolism causing cor causing cor pulmonale is the pulmonale is the sudden increase in sudden increase in pulmonary resistancepulmonary resistance..


In In chronicchronic cor cor pulmonale, pulmonale, right right ventricular ventricular hypertrophyhypertrophy (RVH) (RVH) generally generally predominates.predominates.

InIn acute acute cor cor pulmonale, pulmonale, right right ventricular ventricular dilatationdilatation mainly mainly occurs. occurs.

FrequencyFrequency

Cor pulmonale is estimated to account for 6-7% Cor pulmonale is estimated to account for 6-7% of all types of adult heart disease, with chronic of all types of adult heart disease, with chronic obstructive pulmonary disease (COPD) due to obstructive pulmonary disease (COPD) due to chronic bronchitis or emphysema the causative chronic bronchitis or emphysema the causative factor in more than 50% of cases. factor in more than 50% of cases.

In contrast, acute cor pulmonale is In contrast, acute cor pulmonale is usually secondary to massive pulmonary usually secondary to massive pulmonary embolism. embolism.

Acute massive pulmonary thromboembolism is Acute massive pulmonary thromboembolism is the most common cause of acute life-the most common cause of acute life-threatening cor pulmonale in adults. threatening cor pulmonale in adults.

In the United States, 50,000 deaths are In the United States, 50,000 deaths are estimated to occur per year from pulmonary estimated to occur per year from pulmonary emboli and about half occur within the first emboli and about half occur within the first hour due to acute right heart failure.hour due to acute right heart failure.

FrequencyFrequency

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

Mortality/MorbidityMortality/Morbidity Development of cor pulmonale as a result of Development of cor pulmonale as a result of

a primary pulmonary disease usually heralds a primary pulmonary disease usually heralds a poorer prognosis. For example, patients a poorer prognosis. For example, patients with COPD who develop cor pulmonale have with COPD who develop cor pulmonale have a 30% chance of surviving 5 years. a 30% chance of surviving 5 years.

HistoryHistory

Clinical manifestations of cor pulmonale Clinical manifestations of cor pulmonale generally are generally are nonspecificnonspecific. .

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

The patient may complain of The patient may complain of fatigue, fatigue, tachypnea, exertional dyspnea, and cough. tachypnea, exertional dyspnea, and cough.

Anginal chest painAnginal chest pain also can occur and may be also can occur and may be due to right ventricular ischemia (it usually does due to right ventricular ischemia (it usually does not respond to nitrates) or pulmonary artery not respond to nitrates) or pulmonary artery stretching. stretching.

HistoryHistory

HemoptysisHemoptysis may occur because of rupture of a may occur because of rupture of a dilated or atherosclerotic pulmonary artery. dilated or atherosclerotic pulmonary artery.

Other conditions, such as tumors, bronchiectasis, Other conditions, such as tumors, bronchiectasis, and pulmonary infarction, should be excluded and pulmonary infarction, should be excluded before attributing hemoptysis to pulmonary before attributing hemoptysis to pulmonary hypertension. hypertension.

Rarely, the patient may complain of Rarely, the patient may complain of hoarsenesshoarseness due to compression of the left recurrent laryngeal due to compression of the left recurrent laryngeal nerve by a dilated pulmonary artery. nerve by a dilated pulmonary artery.

A variety of A variety of neurologic symptomsneurologic symptoms may be seen may be seen due to decreased cardiac output and hypoxemia. due to decreased cardiac output and hypoxemia.

HistoryHistory In In advanced stagesadvanced stages, passive , passive hepatic hepatic

congestion secondary to severe congestion secondary to severe right ventricular failureright ventricular failure may lead to may lead to anorexia, right upper quadrant anorexia, right upper quadrant abdominal discomfortabdominal discomfort, and , and jaundice.jaundice.

Syncope with exertionSyncope with exertion, which may be , which may be seen in severe disease, reflects a seen in severe disease, reflects a relative relative inability to increase cardiac inability to increase cardiac output during exerciseoutput during exercise with a with a subsequent subsequent drop in the systemic drop in the systemic arterial pressure. arterial pressure.

HistoryHistory Elevated pulmonary artery pressureElevated pulmonary artery pressure can can

lead to lead to elevated right atrial pressure, elevated right atrial pressure, peripheral venous pressureperipheral venous pressure, and then , and then capillary pressurecapillary pressure and by and by increasing the increasing the hydrostatic gradient,hydrostatic gradient, it leads to it leads to transudation of fluidtransudation of fluid, which appears as , which appears as peripheral edemaperipheral edema. .

A A decrease in glomerular filtration rate decrease in glomerular filtration rate (GFR)(GFR) and and filtration of sodiumfiltration of sodium due to due to hypoxemia play important pathophysiologic hypoxemia play important pathophysiologic roles in this setting and may even roles in this setting and may even have a role have a role for peripheral edemafor peripheral edema in patients with cor in patients with cor pulmonale who have elevated right atrial pulmonale who have elevated right atrial pressure.pressure.

PhysicalPhysical

Physical findingsPhysical findings may reflect may reflect ::- - the underlying lung disease the underlying lung disease - pulmonary hypertension, pulmonary hypertension, - RVHRVH- RV failure. RV failure.

On inspectionOn inspection::- an increase in chest diameter,an increase in chest diameter,- - labored respiratory efforts with retractions of labored respiratory efforts with retractions of

the chest wall,the chest wall,- - distended neck veins with prominent distended neck veins with prominent aa or or vv

waves, waves, - - cyanosis cyanosis

PhysicalPhysical

On auscultationOn auscultation of the lungs of the lungs, , wheezes and wheezes and cracklescrackles may be heard as signs of underlying may be heard as signs of underlying lung disease. lung disease.

On auscultation of the heart:On auscultation of the heart: Splitting of the second heart soundSplitting of the second heart sound with with

accentuation of the pulmonic componentaccentuation of the pulmonic component can be heard in early stages.can be heard in early stages.

A systolic ejection murmurA systolic ejection murmur with with sharp sharp ejection click over the region of the ejection click over the region of the pulmonary arterypulmonary artery may be heard in advanced may be heard in advanced disease, along with a disease, along with a diastolic pulmonary diastolic pulmonary regurgitation murmur. regurgitation murmur.

third and fourth soundsthird and fourth sounds of the heart of the heart systolic murmur of tricuspid regurgitation. systolic murmur of tricuspid regurgitation.

PhysicalPhysical

RVHRVH is characterized by a is characterized by a left parasternal left parasternal or subxiphoid heave.or subxiphoid heave.

Hepatojugular refluxHepatojugular reflux and and pulsatile liverpulsatile liver are are signs of RV failuresigns of RV failure with with systemic systemic venous congestion. venous congestion.

On percussionOn percussion, , hyperresonancehyperresonance of the of the lungs may be a sign of underlying COPD; lungs may be a sign of underlying COPD;

ascitesascites can be seen in severe disease. can be seen in severe disease. Examination of the lower extremities reveals Examination of the lower extremities reveals

evidence of evidence of pitting edemapitting edema. Edema in cor . Edema in cor pulmonale is strongly pulmonale is strongly associated with associated with hypercapniahypercapnia..

CausesCauses

IInvestigate its etiologynvestigate its etiology:: routine laboratory testsroutine laboratory tests chest radiographychest radiography electrocardiographyelectrocardiography Echocardiography Echocardiography Pulmonary function tests may become Pulmonary function tests may become

necessary to confirm the underlying lung necessary to confirm the underlying lung disease. disease.

Ventilation/perfusion (V/Q) scan or chest CT Ventilation/perfusion (V/Q) scan or chest CT scan may be performed if history and physical scan may be performed if history and physical examination suggest pulmonary examination suggest pulmonary thromboembolism thromboembolism

Right heart catheterization is the most Right heart catheterization is the most accurate but invasive test to confirm the accurate but invasive test to confirm the diagnosis of cor pulmonale and gives important diagnosis of cor pulmonale and gives important information regarding the underlying diseases.information regarding the underlying diseases.

CausesCauses

Laboratory investigationsLaboratory investigations :: directed toward directed toward defining the potential underlying etiologies defining the potential underlying etiologies as well as evaluating complications of cor as well as evaluating complications of cor pulmonale. pulmonale.

hematocrit for polycythemiahematocrit for polycythemia (which can (which can be a consequence of underlying lung disease be a consequence of underlying lung disease but can also increase pulmonary arterial but can also increase pulmonary arterial pressure by increasing viscosity), pressure by increasing viscosity),

serum alpha1-antitrypsinserum alpha1-antitrypsin if deficiency is if deficiency is suspected suspected

antinuclear antibody levelantinuclear antibody level for collagen for collagen vascular disease such as scleroderma. vascular disease such as scleroderma.

Hypercoagulability statesHypercoagulability states can be can be evaluated by serum levels of proteins S and evaluated by serum levels of proteins S and C, antithrombin III, factor V Leyden, C, antithrombin III, factor V Leyden, anticardiolipin antibodies, and homocysteine. anticardiolipin antibodies, and homocysteine.

CausesCauses

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

Elevated (BNP) levelElevated (BNP) level alone is alone is not not adequate to establishadequate to establish presence of presence of cor pulmonalecor pulmonale, but it helps to diagnose , but it helps to diagnose cor pulmonale in conjunction with other cor pulmonale in conjunction with other noninvasive tests and in appropriate noninvasive tests and in appropriate clinical settings. clinical settings.

Imaging StudiesImaging Studies

Chest roentgenographyChest roentgenography: may show : may show enlargement of the central pulmonary arteries enlargement of the central pulmonary arteries with oligemic peripheral lung fields. with oligemic peripheral lung fields.

Pulmonary hypertensionPulmonary hypertension should be should be suspected when the suspected when the right descending right descending pulmonary artery is larger than 16 mm in pulmonary artery is larger than 16 mm in diameter and the left pulmonary artery is diameter and the left pulmonary artery is larger than 18 mm in diameter. larger than 18 mm in diameter.

Right ventricular enlargementRight ventricular enlargement leads to an leads to an increase of the transverse diameter of the increase of the transverse diameter of the heartheart shadow to the right on the shadow to the right on the posteroanterior view and filling of the posteroanterior view and filling of the retrosternal air space on the lateral view. retrosternal air space on the lateral view.

Imaging StudiesImaging Studies

EchocardiographyEchocardiography: : usually demonstrates signs of usually demonstrates signs of chronic RV pressure chronic RV pressure

overload.overload. As this overload progresses, As this overload progresses, increased increased thickness of the RV wall with paradoxical motion thickness of the RV wall with paradoxical motion of the interventricular septum during systole of the interventricular septum during systole occurs. occurs.

At an advanced stage, At an advanced stage, RV dilatationRV dilatation occurs and the occurs and the septum shows abnormal diastolic flattening. septum shows abnormal diastolic flattening.

In extreme cases, In extreme cases, the septum may actually bulge the septum may actually bulge into the left ventricular cavity during diastole into the left ventricular cavity during diastole resulting in decreased diastolic volume of LV and resulting in decreased diastolic volume of LV and reduction of LV output.reduction of LV output.

Doppler echocardiography Doppler echocardiography :: used to used to estimate estimate pulmonary arterial pressure, taking advantage of pulmonary arterial pressure, taking advantage of the functional tricuspid insufficiency that is the functional tricuspid insufficiency that is usually present in pulmonary hypertensionusually present in pulmonary hypertension. . Doppler echocardiography is considered the most Doppler echocardiography is considered the most reliable noninvasive technique to estimate pulmonary reliable noninvasive technique to estimate pulmonary artery pressure. artery pressure.

Imaging studiesImaging studies

Ventilation/perfusion (V/Q) lung Ventilation/perfusion (V/Q) lung scanningscanning, , pulmonary angiographypulmonary angiography, and , and chest CT scanningchest CT scanning may be indicated to may be indicated to diagnose pulmonary thromboembolism as diagnose pulmonary thromboembolism as the underlying etiology of cor pulmonale. the underlying etiology of cor pulmonale.

Pulmonary thromboembolism has a wide Pulmonary thromboembolism has a wide range of clinical presentations from massive range of clinical presentations from massive embolism with acute and severe embolism with acute and severe hemodynamic instability to multiple chronic hemodynamic instability to multiple chronic peripheral embolisms that may present with peripheral embolisms that may present with cor pulmonale. cor pulmonale.

Magnetic resonance imaging (MRIMagnetic resonance imaging (MRI) of the ) of the heart is another modality that can provide heart is another modality that can provide valuable information about RV mass, septal valuable information about RV mass, septal flattening, and ventricular function.flattening, and ventricular function.

Other TestsOther Tests

Electrocardiography (ECG)Electrocardiography (ECG) abnormalities in cor pulmonale reflect the abnormalities in cor pulmonale reflect the presence of RVH, RV strain, or underlying presence of RVH, RV strain, or underlying pulmonary disease. These pulmonary disease. These electrocardiographic changes may include the electrocardiographic changes may include the following:following:

Right axis deviationRight axis deviation P-pulmonale pattern (an increase in P P-pulmonale pattern (an increase in P

wave amplitude in leads 2, 3, and aVF) wave amplitude in leads 2, 3, and aVF) S1Q3T3 patternS1Q3T3 pattern and and incomplete (or incomplete (or

complete) right bundle branch block, complete) right bundle branch block, especially if pulmonary embolism is the especially if pulmonary embolism is the underlying etiology underlying etiology

Low-voltage QRSLow-voltage QRS because of underlying because of underlying COPD with hyperinflation COPD with hyperinflation

ElectrocardiographyElectrocardiography

ElectrocardiographyElectrocardiography

RRhythm disturbanceshythm disturbances may be present in chronic cor may be present in chronic cor pulmonalepulmonale::

isolated premature atrial depolarizations isolated premature atrial depolarizations supraventricular tachycardias, including paroxysmal supraventricular tachycardias, including paroxysmal

atrial tachycardia, multifocal atrial tachycardia, atrial atrial tachycardia, multifocal atrial tachycardia, atrial fibrillation, atrial flutter, and junctional tachycardia.fibrillation, atrial flutter, and junctional tachycardia.

These dysrhythmias may be triggered by processes These dysrhythmias may be triggered by processes secondary to the underlying disease, (eg, anxiety, secondary to the underlying disease, (eg, anxiety, hypoxemia, acid-base imbalance, electrolyte hypoxemia, acid-base imbalance, electrolyte disturbances, excessive use of bronchodilators, disturbances, excessive use of bronchodilators, heightened sympathetic activity). heightened sympathetic activity).

Life-threatening ventricular tachyarrhythmias are less Life-threatening ventricular tachyarrhythmias are less common.common.

ProceduresProcedures

Cardiac catheterizationCardiac catheterization:: Right-heart catheterization is considered the Right-heart catheterization is considered the

most precise method for diagnosis and most precise method for diagnosis and quantification of pulmonary hypertension.quantification of pulmonary hypertension.

It is indicated when echocardiography It is indicated when echocardiography cannot assess the severity of a tricuspid cannot assess the severity of a tricuspid regurgitant jet, thus excluding an regurgitant jet, thus excluding an assessment of pulmonary hypertension.assessment of pulmonary hypertension.

Lung biopsy may occasionally be indicated to Lung biopsy may occasionally be indicated to determine underlying etiology.determine underlying etiology.

Medical CareMedical Care

Cardiopulmonary support for patients Cardiopulmonary support for patients experiencing acute cor pulmonale with resultant experiencing acute cor pulmonale with resultant acute RV failure includes acute RV failure includes fluid loadingfluid loading and and vasoconstrictvasoconstrictiioonn (eg, epinephrin) administration (eg, epinephrin) administration to maintain adequate blood pressure. to maintain adequate blood pressure.

primary problem should be corrected, if primary problem should be corrected, if possiblepossible(( for massive pulmonary embolism, for massive pulmonary embolism, consider administration of anticoagulation, consider administration of anticoagulation, thrombolytic agents or surgical embolectomythrombolytic agents or surgical embolectomy;; consider bronchodilation and infection treatment consider bronchodilation and infection treatment in patients with COPDin patients with COPD))

Oxygen therapy, diuretics, vasodilators, digitalis, Oxygen therapy, diuretics, vasodilators, digitalis, theophylline, and anticoagulation therapy are all theophylline, and anticoagulation therapy are all different modalities used in the long-term different modalities used in the long-term management of chronic cor pulmonale.management of chronic cor pulmonale.

Oxygen therapyOxygen therapy

Oxygen therapy is of great importance in Oxygen therapy is of great importance in patients with underlying COPD, particularly when patients with underlying COPD, particularly when administered on a continuous basis. With cor administered on a continuous basis. With cor pulmonale, the partial pressure of oxygen (PO2) pulmonale, the partial pressure of oxygen (PO2) is likely to be below 55 mm Hg and decreases is likely to be below 55 mm Hg and decreases further with exercise and during sleep.further with exercise and during sleep.

Oxygen therapy relieves hypoxemic pulmonary Oxygen therapy relieves hypoxemic pulmonary vasoconstriction, which then improves cardiac vasoconstriction, which then improves cardiac output, lessens sympathetic vasoconstriction, output, lessens sympathetic vasoconstriction, alleviates tissue hypoxemia, and improves renal alleviates tissue hypoxemia, and improves renal perfusion. perfusion.

In general, in patients with COPD, long-term In general, in patients with COPD, long-term oxygen therapy is recommended when PaO2 is oxygen therapy is recommended when PaO2 is less than 55 mm Hg or O2 saturation is less than less than 55 mm Hg or O2 saturation is less than 88%. 88%.

DiureticsDiuretics Diuretics are used in the management of Diuretics are used in the management of

chronic cor pulmonale, particularly when the chronic cor pulmonale, particularly when the right ventricular filling volume is markedly right ventricular filling volume is markedly elevated and in the management of elevated and in the management of associated peripheral edema.associated peripheral edema.

Diuretics may result in improvement of the Diuretics may result in improvement of the function of both the right and left ventricles;function of both the right and left ventricles;

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

The adverse electrolyte and acid-base effect The adverse electrolyte and acid-base effect of diuretic use can also lead to cardiac of diuretic use can also lead to cardiac arrhythmia, which can diminish cardiac output. arrhythmia, which can diminish cardiac output. Therefore, diuresis, while recommended in the Therefore, diuresis, while recommended in the management of chronic cor pulmonale, needs management of chronic cor pulmonale, needs to be used with great caution. to be used with great caution.

Vasodilator drugsVasodilator drugs

Vasodilator drugs have been advocated in Vasodilator drugs have been advocated in the long-term management of chronic cor the long-term management of chronic cor pulmonale with pulmonale with modest results.modest results.

Calcium channel blockers, particularly oral Calcium channel blockers, particularly oral sustained-release nifedipine and diltiazem, sustained-release nifedipine and diltiazem, can lower pulmonary pressures, although can lower pulmonary pressures, although they appear more effective in primary rather they appear more effective in primary rather than secondary pulmonary hypertension.than secondary pulmonary hypertension.

Other classes of vasodilators, such as beta Other classes of vasodilators, such as beta agonists, nitrates, and angiotensin-agonists, nitrates, and angiotensin-converting enzyme (ACE) inhibitors have converting enzyme (ACE) inhibitors have been tried but, in general, vasodilators have been tried but, in general, vasodilators have failed to show sustained benefit in patients failed to show sustained benefit in patients with COPD and they are not routinely used. with COPD and they are not routinely used.

Beta-selective agonistsBeta-selective agonists Beta-selective agonists have an additional advantage of Beta-selective agonists have an additional advantage of

bronchodilator and mucociliary clearance effect. bronchodilator and mucociliary clearance effect.

The Food and Drug Administration (FDA) has approved The Food and Drug Administration (FDA) has approved epoprostenol, treprostinil, bosentan, and iloprost for epoprostenol, treprostinil, bosentan, and iloprost for treatment of primary pulmonary hypertension.treatment of primary pulmonary hypertension.

Epoprostenol, treprostinil, and iloprost are Epoprostenol, treprostinil, and iloprost are prostacyclin (PGI2) analogues and have potent prostacyclin (PGI2) analogues and have potent vasodilatory properties.vasodilatory properties.

BosentanBosentan is a mixed endothelin-A and endothelin-B is a mixed endothelin-A and endothelin-B receptor antagonist receptor antagonist indicated for pulmonary arterial indicated for pulmonary arterial hypertension (PAH), including primary pulmonary hypertension (PAH), including primary pulmonary hypertension (PPHhypertension (PPH). In clinical trials, it improved exercise ). In clinical trials, it improved exercise capacity, decreased rate of clinical deterioration, and capacity, decreased rate of clinical deterioration, and improved hemodynamics.improved hemodynamics.

The PDE5 inhibitor sildenafilThe PDE5 inhibitor sildenafil has also been intensively has also been intensively studied and approved by the FDA for treatment of studied and approved by the FDA for treatment of pulmonary hypertension based on a large randomized study. pulmonary hypertension based on a large randomized study. Sildenafil promotes selective smooth muscle relaxation in Sildenafil promotes selective smooth muscle relaxation in lung vasculature.lung vasculature.

Cardiac glycosidesCardiac glycosides The use of cardiac glycosides, such as digitalis, The use of cardiac glycosides, such as digitalis,

in patients with cor pulmonale has been in patients with cor pulmonale has been controversial, and the beneficial effect of these controversial, and the beneficial effect of these drugs is not as obvious as in the setting of left drugs is not as obvious as in the setting of left heart failure.heart failure.

Nevertheless, studies have confirmed a modest Nevertheless, studies have confirmed a modest effect of digitalis on the failing right ventricle in effect of digitalis on the failing right ventricle in patients with chronic cor pulmonale.patients with chronic cor pulmonale.

It must be used cautiously, however, and It must be used cautiously, however, and should not be used during the acute phases of should not be used during the acute phases of respiratory insufficiency when large fluctuations respiratory insufficiency when large fluctuations in levels of hypoxia and acidosis may occur. in levels of hypoxia and acidosis may occur.

Patients with hypoxemia or acidosis are at Patients with hypoxemia or acidosis are at increased risk of developing arrhythmias due to increased risk of developing arrhythmias due to digitalis through different mechanisms including digitalis through different mechanisms including sympathoadrenal stimulation. sympathoadrenal stimulation.

TheophyllineTheophylline

In addition to bronchodilatory effect, In addition to bronchodilatory effect, theophylline has been reported to reduce theophylline has been reported to reduce pulmonary vascular resistance and pulmonary pulmonary vascular resistance and pulmonary arterial pressures acutely in patients with arterial pressures acutely in patients with chronic cor pulmonale secondary to COPD. chronic cor pulmonale secondary to COPD.

Theophylline has a weak inotropic effect and Theophylline has a weak inotropic effect and thus may improve right and left ventricular thus may improve right and left ventricular ejection. As a result, considering the use of ejection. As a result, considering the use of theophylline as adjunctive therapy in the theophylline as adjunctive therapy in the management of chronic or decompensated management of chronic or decompensated cor pulmonale is reasonable in patients with cor pulmonale is reasonable in patients with underlying COPD. underlying COPD.

WarfarinWarfarin

Anticoagulation with warfarin is recommended in Anticoagulation with warfarin is recommended in patients at high risk for thromboembolism.patients at high risk for thromboembolism.

The beneficial role of anticoagulation in The beneficial role of anticoagulation in improving the symptoms and mortality in improving the symptoms and mortality in patients with primary pulmonary arterial patients with primary pulmonary arterial hypertension clearly was demonstrated in a hypertension clearly was demonstrated in a variety of clinical trials. variety of clinical trials.

The evidence of benefit, however, has not been The evidence of benefit, however, has not been established in patients with secondary established in patients with secondary pulmonary arterial hypertension. pulmonary arterial hypertension.

Therefore, anticoagulation therapy may be used Therefore, anticoagulation therapy may be used in patients with cor pulmonale secondary to in patients with cor pulmonale secondary to thromboembolic phenomena and with underlying thromboembolic phenomena and with underlying primary pulmonary arterial hypertension. primary pulmonary arterial hypertension.

Surgical CareSurgical Care

PhlebotomyPhlebotomy is indicated in patients with chronic cor is indicated in patients with chronic cor pulmonale and chronic hypoxia causing severe pulmonale and chronic hypoxia causing severe polycythemia, defined as hematocrit of 65 or more.polycythemia, defined as hematocrit of 65 or more.

Phlebotomy results in a decrease in mean pulmonary Phlebotomy results in a decrease in mean pulmonary artery pressure, a decrease in mean pulmonary artery pressure, a decrease in mean pulmonary vascular resistance, and an improvement in exercise vascular resistance, and an improvement in exercise performance in such patients. performance in such patients.

Generally, phlebotomy should be reserved as an Generally, phlebotomy should be reserved as an adjunctive therapy for patients with acute adjunctive therapy for patients with acute decompensation of cor pulmonale and patients who decompensation of cor pulmonale and patients who remain significantly polycythemic despite appropriate remain significantly polycythemic despite appropriate long-term oxygen therapy. long-term oxygen therapy.

Replacement of the acute volume loss with a saline Replacement of the acute volume loss with a saline infusion may be necessary to avoid important infusion may be necessary to avoid important decreases in systemic blood pressure. decreases in systemic blood pressure.

Surgical CareSurgical Care

No surgical treatment exists for most diseases that No surgical treatment exists for most diseases that cause chronic cor pulmonale.cause chronic cor pulmonale.

Pulmonary embolectomy is efficacious for unresolved Pulmonary embolectomy is efficacious for unresolved pulmonary emboli, which contribute to pulmonary pulmonary emboli, which contribute to pulmonary hypertension. hypertension.

Single-lung, double-lung, and heart-lung Single-lung, double-lung, and heart-lung transplantation are all used to salvage the terminal transplantation are all used to salvage the terminal phases of several diseases (eg, primary pulmonary phases of several diseases (eg, primary pulmonary hypertension, emphysema, idiopathic pulmonary hypertension, emphysema, idiopathic pulmonary fibrosis, cystic fibrosis) complicated by cor pulmonale.fibrosis, cystic fibrosis) complicated by cor pulmonale.

Apparently, lung transplantation will lead to a Apparently, lung transplantation will lead to a reversal of right ventricular dysfunction from the reversal of right ventricular dysfunction from the chronic stress of pulmonary hypertension. However, chronic stress of pulmonary hypertension. However, strict selection criteria for lung transplant recipients strict selection criteria for lung transplant recipients must be met because of the limited availability of must be met because of the limited availability of organ donors.organ donors.

DiureticsDiuretics Are used to decrease the elevated right ventricular filling Are used to decrease the elevated right ventricular filling

volume in patients with chronic cor pulmonale.volume in patients with chronic cor pulmonale. Drug NameDrug Name: : Furosemide (Lasix)Furosemide (Lasix) DescriptionDescription:: Furosemide is a powerful loop diuretic that Furosemide is a powerful loop diuretic that

works on thick ascending limb of Henle loop, causing a works on thick ascending limb of Henle loop, causing a reversible block in reabsorption of sodium, potassium, and reversible block in reabsorption of sodium, potassium, and chloride.chloride.

Adult DoseAdult Dose: : 20-80 mg/d PO/IV/IM; may titrate to maximum 20-80 mg/d PO/IV/IM; may titrate to maximum dose of 600 mg/ddose of 600 mg/d

ContraindicationsContraindications: : Documented hypersensitivity; hepatic Documented hypersensitivity; hepatic coma; anuria; concurrent severe electrolyte depletioncoma; anuria; concurrent severe electrolyte depletion

InteractionInteractions:s: auditory toxicity appears to be increased with auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken activity of warfarin may be enhanced when taken concurrently with this medication; concurrently with this medication;

PrecautionsPrecautions: : Perform frequent serum electrolyte, carbon Perform frequent serum electrolyte, carbon dioxide, glucose, creatinine, uric acid, calcium, and BUN dioxide, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and determinations during first few months of therapy and periodically thereafterperiodically thereafter

Calcium channel blockersCalcium channel blockers

These agents inhibit movement of calcium ions across the cell These agents inhibit movement of calcium ions across the cell membrane, depressing both impulse formation (automaticity) membrane, depressing both impulse formation (automaticity) and conduction velocity.and conduction velocity.

Drug NameDrug Name: : NifedipineNifedipine (Procardia) (Procardia) DescriptionDescription: : Especially in the sustained-release form, Especially in the sustained-release form,

nifedipine is a calcium channel blocker that has proven to be nifedipine is a calcium channel blocker that has proven to be fairly effective in the management of chronic cor pulmonale fairly effective in the management of chronic cor pulmonale caused by primary pulmonary hypertension.caused by primary pulmonary hypertension.

Sublingual administration generally is safe, despite Sublingual administration generally is safe, despite theoretical concerns.theoretical concerns.

Adult DoseAdult Dose::10-30 mg SR cap PO tid; not to exceed 120-180 10-30 mg SR cap PO tid; not to exceed 120-180 mg/dmg/d30-60 mg SR tab PO qd; not to exceed 90-120 mg/30-60 mg SR tab PO qd; not to exceed 90-120 mg/dayday

ContraindicationsContraindications: : Documented hypersensitivityDocumented hypersensitivity InteractionsInteractions: : Monitor oral anticoagulants when used Monitor oral anticoagulants when used

concomitantly; coadministration with any agent that can concomitantly; coadministration with any agent that can lower BP, including beta-blockers and opioids, can result in lower BP, including beta-blockers and opioids, can result in severe hypotension; H2 blockers (cimetidine) may increase severe hypotension; H2 blockers (cimetidine) may increase toxicitytoxicity

PrecautionsPrecautions::Aortic stenosis; angina; congestive heart failure; may cause Aortic stenosis; angina; congestive heart failure; may cause lower extremity edema; lower extremity edema;

Cardiac glycosidesCardiac glycosides These agents decrease AV nodal conduction primarily by These agents decrease AV nodal conduction primarily by

increasing vagal tone.increasing vagal tone. Drug NameDrug Name: : Digoxin (Lanoxin)Digoxin (Lanoxin) DescriptionDescription::

Has a positive inotropic effect on failing myocardium. Has a positive inotropic effect on failing myocardium. Effect is achieved via inhibition of the Na+/K+-ATPase Effect is achieved via inhibition of the Na+/K+-ATPase pump, leading to increase in intracellular sodium pump, leading to increase in intracellular sodium concentration along with concomitant increase in concentration along with concomitant increase in intracellular calcium concentration by means of intracellular calcium concentration by means of calcium-sodium exchange mechanism. Net result is calcium-sodium exchange mechanism. Net result is augmentation of myocardial contractility.augmentation of myocardial contractility.

Adult DoseAdult Dose: : 0.125-0.375 mg PO qd; may be 0.125-0.375 mg PO qd; may be administered qod; available in PO/IV/IM preparationsadministered qod; available in PO/IV/IM preparations

ContraindicationsContraindications: : Documented hypersensitivity; Documented hypersensitivity; beriberi heart disease; idiopathic hypertrophic beriberi heart disease; idiopathic hypertrophic subaortic stenosis; constrictive pericarditis; carotid subaortic stenosis; constrictive pericarditis; carotid sinus syndromesinus syndrome

Cardiac glycosidesCardiac glycosidesInteractionsInteractions:: Medications that may increase digoxin levelsMedications that may increase digoxin levels

include alprazolam, benzodiazepines, bepridil, captopril, include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, oral amiodarone, diltiazem, aminoglycosides, oral amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil;tetracycline, tolbutamide, and verapamil;

MMedications that may decrease serum digoxin edications that may decrease serum digoxin levelslevels include aminoglutethimide, antihistamines, include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, cholestyramine, neomycin, penicillamine, aminoglycosides, oral colestipol, hydantoins, aminoglycosides, oral colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment hypoglycemic agents, antineoplastic treatment combinations (eg, carmustine, bleomycin, methotrexate, combinations (eg, carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acidkaolin/pectin, and aminosalicylic acid

Cardiac glycosidesCardiac glycosides

PrecautionsPrecautions:: Hypokalemia may reduce positive inotropic effect of Hypokalemia may reduce positive inotropic effect of

digitalis; digitalis; IV calcium may produce arrhythmias in digitalized IV calcium may produce arrhythmias in digitalized

patients;patients; hypercalcemia predisposes patient to digitalis toxicity; hypercalcemia predisposes patient to digitalis toxicity; hypocalcemia can make digoxin ineffective until serum hypocalcemia can make digoxin ineffective until serum

calcium levels are normal; calcium levels are normal; magnesium replacement therapy must be instituted in magnesium replacement therapy must be instituted in

patients with hypomagnesemia to prevent digitalis patients with hypomagnesemia to prevent digitalis toxicity; toxicity;

patients diagnosed with incomplete AV block may patients diagnosed with incomplete AV block may progress to complete block when treated with digoxin; progress to complete block when treated with digoxin;

exercise caution in hypothyroidism, hypoxia, and acute exercise caution in hypothyroidism, hypoxia, and acute myocarditismyocarditis

AnticoagulantsAnticoagulants

These agents may reduce incidence of embolisms when These agents may reduce incidence of embolisms when used fast, effectively, and early.used fast, effectively, and early.

Drug NameDrug Name: : Warfarin (Coumadin)Warfarin (Coumadin) DescriptionDescription: : Most commonly used oral Most commonly used oral

anticoagulant. Interferes with hepatic synthesis of anticoagulant. Interferes with hepatic synthesis of vitamin K-dependent coagulation factors. Used for vitamin K-dependent coagulation factors. Used for prophylaxis and treatment of venous thrombosis, prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders.pulmonary embolism, and thromboembolic disorders.

Adult DoseAdult Dose: : 2-10 mg/d PO2-10 mg/d PO// qd; adjust dose to an INR qd; adjust dose to an INR of 1.5:2 or higher depending on the condition of 1.5:2 or higher depending on the condition requiring anticoagulationrequiring anticoagulation

ContraindicationsContraindications: : Documented hypersensitivity; Documented hypersensitivity; severe liver or kidney disease; open wounds; GI ulcerssevere liver or kidney disease; open wounds; GI ulcers

AnticoagulantsAnticoagulants

InteractionsInteractions: : Griseofulvin, carbamazepine, glutethimide, Griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate may decrease anticoagulant contraceptives, and sucralfate may decrease anticoagulant effects; oral antibiotics, phenylbutazone, salicylates, effects; oral antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac may increase anticoagulant effectsand sulindac may increase anticoagulant effects

PregnancyPregnancy:: - Fetal risk shown in humans; - Fetal risk shown in humans;

PrecautionsPrecautions: : Dose needs to be adjusted to INR; caution in Dose needs to be adjusted to INR; caution in bleeding tendency and hazardous active hemorrhagic bleeding tendency and hazardous active hemorrhagic conditions, malignant hypertension, patients at high risk of conditions, malignant hypertension, patients at high risk of recurrent trauma, (eg, people with alcoholism or psychosis, recurrent trauma, (eg, people with alcoholism or psychosis, unsupervised patients who are senile); warfarin anaphylaxis, unsupervised patients who are senile); warfarin anaphylaxis, hepatic, renal, thyroid, allergic, and hematologic hepatic, renal, thyroid, allergic, and hematologic hypocoagulable conditions and disorders; caution in active hypocoagulable conditions and disorders; caution in active tuberculosis tuberculosis

MethylxanthinesMethylxanthines

Potentiate exogenous catecholamines and stimulate Potentiate exogenous catecholamines and stimulate endogenous catecholamine release and diaphragmatic endogenous catecholamine release and diaphragmatic muscular relaxation, which, in turn, stimulates muscular relaxation, which, in turn, stimulates bronchodilation.bronchodilation.

Drug NameDrug Name::TheophyllineTheophylline (Aminophyllin, Theo-24, (Aminophyllin, Theo-24, Theolair, Theo-Dur)Theolair, Theo-Dur)

DescriptionDescription: : Mechanism of action is not well defined Mechanism of action is not well defined yet. Was formerly thought that this drug increases yet. Was formerly thought that this drug increases intracellular cyclic AMP by causing inhibition of intracellular cyclic AMP by causing inhibition of phosphodiesterase; however, current data do not phosphodiesterase; however, current data do not support that.support that.

DoseDose: : Loading dose: 5.6 mg/kg IV over 20 min Loading dose: 5.6 mg/kg IV over 20 min Maintenance dose: IV infusion at 0.5-0.7 mg/kg/h; also Maintenance dose: IV infusion at 0.5-0.7 mg/kg/h; also

available in oral preparationavailable in oral preparation

MethylxanthinesMethylxanthines

ContraindicationsContraindications: : Documented hypersensitivity; Documented hypersensitivity; uncontrolled arrhythmias; peptic ulcers; uncontrolled arrhythmias; peptic ulcers; hyperthyroidism; uncontrolled seizure disordershyperthyroidism; uncontrolled seizure disorders

InteractionsInteractions: : Effects may decrease with Effects may decrease with aminoglutethimide, barbiturates, carbamazepine, aminoglutethimide, barbiturates, carbamazepine, ketoconazole, loop diuretics, charcoal, hydantoins, ketoconazole, loop diuretics, charcoal, hydantoins, phenobarbital, phenytoin, rifampin, isoniazid, and phenobarbital, phenytoin, rifampin, isoniazid, and sympathomimetics; effects may increase with sympathomimetics; effects may increase with allopurinol, beta-blockers, ciprofloxacin, allopurinol, beta-blockers, ciprofloxacin, corticosteroids, disulfiram, quinolones, thyroid corticosteroids, disulfiram, quinolones, thyroid hormones, ephedrine, carbamazepine, cimetidine, hormones, ephedrine, carbamazepine, cimetidine, erythromycin, macrolides, propranolol, and interfererythromycin, macrolides, propranolol, and interferonon

PrecautionsPrecautions: : Has low serum therapeutic-to-toxicity Has low serum therapeutic-to-toxicity ratio, and, therefore, serum level monitoring is ratio, and, therefore, serum level monitoring is important; peptic ulcer; hypertension; important; peptic ulcer; hypertension; tachyarrhythmias; hyperthyroidism; compromised tachyarrhythmias; hyperthyroidism; compromised cardiac function; do not inject IV solution faster than cardiac function; do not inject IV solution faster than 25 mg/min; patients diagnosed with pulmonary edema 25 mg/min; patients diagnosed with pulmonary edema or liver dysfunction are at greater risk of toxicity or liver dysfunction are at greater risk of toxicity because of reduced drug clearancebecause of reduced drug clearance..

Endothelin receptor Endothelin receptor antagonistsantagonists

Competitively bind to endothelin-1 (ET-1) receptors ETA and Competitively bind to endothelin-1 (ET-1) receptors ETA and ETB causing reduction in pulmonary artery pressure (PAP), ETB causing reduction in pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR), and mean right atrial pulmonary vascular resistance (PVR), and mean right atrial pressure (RAP).pressure (RAP).

Drug NameDrug Name: : Bosentan (Tracleer)Bosentan (Tracleer) DescriptionDescription: : Endothelin receptor antagonist indicated for Endothelin receptor antagonist indicated for

the treatment of pulmonary arterial hypertension in the treatment of pulmonary arterial hypertension in patients with WHO Class III or IV symptoms, to improve patients with WHO Class III or IV symptoms, to improve exercise ability and decrease rate of clinical worsening. exercise ability and decrease rate of clinical worsening. Inhibits vessel constriction and elevation of blood pressure Inhibits vessel constriction and elevation of blood pressure by competitively binding to endothelin-1 (ET-1) receptors by competitively binding to endothelin-1 (ET-1) receptors ETA and ETB in endothelium and vascular smooth muscle. ETA and ETB in endothelium and vascular smooth muscle. This leads to significant increase in cardiac index (CI) This leads to significant increase in cardiac index (CI) associated with significant reduction in pulmonary artery associated with significant reduction in pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR), and pressure (PAP), pulmonary vascular resistance (PVR), and mean right atrial pressure (RAP). mean right atrial pressure (RAP). ::

DoseDose: : <40 kg: 62.5 mg PO bid; not to exceed 125 mg/d<40 kg: 62.5 mg PO bid; not to exceed 125 mg/d>40 kg: 62.5 mg PO bid for 4 wk initially, then increase to >40 kg: 62.5 mg PO bid for 4 wk initially, then increase to 125 mg PO bid125 mg PO bid

Endothelin receptor Endothelin receptor antagonistsantagonists

ContraindicationsContraindications: : Documented hypersensitivity; Documented hypersensitivity; InteractionsInteractions: : Toxicity may increase when administered Toxicity may increase when administered

concomitantly with inhibitors of isoenzymes CYP450 2C9 and concomitantly with inhibitors of isoenzymes CYP450 2C9 and CYP450 3A4 (eg, ketoconazole, erythromycin, fluoxetine, sertraline, CYP450 3A4 (eg, ketoconazole, erythromycin, fluoxetine, sertraline, amiodarone, and cyclosporine A); induces isoenzymes CYP450 2C9 amiodarone, and cyclosporine A); induces isoenzymes CYP450 2C9 and CYP450 3A4 causing decrease in plasma concentrations of and CYP450 3A4 causing decrease in plasma concentrations of drugs metabolized by these enzymes including glyburide as well as drugs metabolized by these enzymes including glyburide as well as other hypoglycemics, cyclosporine A, hormonal contraceptives, other hypoglycemics, cyclosporine A, hormonal contraceptives, simvastatin, and possibly other statins; hepatotoxicity increases simvastatin, and possibly other statins; hepatotoxicity increases with concomitant administration of glyburidewith concomitant administration of glyburide

PregnancyPregnancy:: - Contraindicated; benefit does not outweigh risk - Contraindicated; benefit does not outweigh riskPrecautionsPrecautions: : Causes at least 3-fold elevation of liver Causes at least 3-fold elevation of liver aminotransferases (ie, ALT, AST) in about 11% of patients; may aminotransferases (ie, ALT, AST) in about 11% of patients; may elevate bilirubin (serum aminotransferase levels must be measured elevate bilirubin (serum aminotransferase levels must be measured prior to initiation of treatment and then monthly); caution in prior to initiation of treatment and then monthly); caution in patients with mildly impaired liver function (avoid in patients with patients with mildly impaired liver function (avoid in patients with moderate or severe liver impairment); not recommended while moderate or severe liver impairment); not recommended while breastfeeding; monitor hemoglobin levels after 1 and 3 mo of breastfeeding; monitor hemoglobin levels after 1 and 3 mo of treatment and every 3 mo thereafter; exclude pregnancy before treatment and every 3 mo thereafter; exclude pregnancy before initiating treatment and prevent thereafter by use of reliable initiating treatment and prevent thereafter by use of reliable contraception; headache and nasopharyngitis may occurcontraception; headache and nasopharyngitis may occur

PrognosisPrognosis

The prognosis of cor pulmonale is variable The prognosis of cor pulmonale is variable depending upon underlying pathology. depending upon underlying pathology.

Patients with cor pulmonale due to COPD Patients with cor pulmonale due to COPD have a high 2-year mortality.have a high 2-year mortality.

Patient EducationPatient Education Patient education regarding the Patient education regarding the

importance of adherence to medical importance of adherence to medical therapy is vital because appropriate therapy is vital because appropriate treatment of both hypoxia and underlying treatment of both hypoxia and underlying medical illness can improve mortality and medical illness can improve mortality and morbidity.morbidity.

Documents

9. Cor Pulmonale