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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]
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.
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.
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.
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.
Signs
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.
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]
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
Arterial blood gas measurements may provide important information about the level
of oxygenation and type of acid-base disorder.
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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
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.
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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
Cardiac Catheterization
Lung Biopsy
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.
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Patient education
Complications
Oxygen Therapy
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]
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.
Vasodilator drugs
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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.
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.
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
Warfarin
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.
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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.
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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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.
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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