eMedicine Specialties > Cardiology > Myocardial Disease and Cardiomyopathies

Cor Pulmonale: Differential Diagnoses & Workup

Author: Ali A Sovari, MD, Research Fellow, Division of Cardiology, David Geffen School of Medicine, University of California, Los Angeles (UCLA)
Coauthor(s): Ravi H Dave, MD, Associate Professor of Medicine, University of California at Los Angeles David Geffen School of Medicine; Abraham G Kocheril, MD, FACC, FACP, Professor of Medicine, Director of Clinical Electrophysiology, University of Illinois at Chicago
Contributor Information and Disclosures

Updated: Sep 3, 2008

Differential Diagnoses

Ventricular Septal Defect

Other Problems to Be Considered

Congestive (biventricular) heart failure
Primary pulmonic stenosis
Primary pulmonary hypertension
Right-sided heart failure due to congenital heart diseases
Right heart failure due to right ventricular infarction

Workup

Laboratory Studies

  • 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. Pulmonary function tests may become necessary to confirm the underlying lung disease. Ventilation/perfusion (V/Q) scan or chest CT scan may be performed if history and physical examination suggest pulmonary thromboembolism as the cause or if other diagnostic tests do not suggest other etiologies. 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. Any abnormal result in each of these tests may need further diagnostic evaluation in that specific direction.
  • Laboratory investigations are directed toward defining the potential underlying etiologies as well as evaluating complications of cor pulmonale. In specific instances, appropriate lab studies may include the following: hematocrit for polycythemia (which can be a consequence of underlying lung disease but can also increase pulmonary arterial pressure by increasing viscosity), serum alpha1-antitrypsin if deficiency is suspected, and antinuclear antibody level for collagen vascular disease such as scleroderma. Hypercoagulability states can be evaluated by serum levels of proteins S and C, antithrombin III, factor V Leyden, anticardiolipin antibodies, and homocysteine.
  • Arterial blood gas tests may provide important information about the level of oxygenation and type of acid-base disorder.
  • Elevated brain natriuretic peptide (BNP) level alone is not adequate to establish 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.

Imaging Studies

Imaging studies may show evidence of underlying cardiopulmonary diseases, pulmonary hypertension, or its consequence, right ventricular enlargement.

  • Chest roentgenography: 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.
  • Echocardiography: Two-dimensional echocardiography usually demonstrates signs of chronic 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 cavity during diastole resulting in decreased diastolic volume of LV and reduction of LV output.

    Doppler echocardiography is now used to estimate pulmonary arterial pressure, taking advantage of the functional tricuspid insufficiency that is usually present in pulmonary hypertension. Doppler echocardiography is considered the most reliable noninvasive technique to estimate pulmonary artery pressure. 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.
  • Ventilation/perfusion (V/Q) lung scanning, pulmonary angiography, and chest CT scanning may be indicated to diagnose pulmonary thromboembolism as the underlying etiology of cor pulmonale. These tests may be performed early in the diagnostic workup if any evidence of pulmonary embolism appears in history and physical examination. The test may also be considered later in the workup if other tests are not suggestive of any other etiology. Pulmonary thromboembolism has a wide range of clinical presentations from massive embolism with acute and severe hemodynamic instability to multiple chronic peripheral embolisms that may present with cor pulmonale.
  • Ultrafast, ECG-gated CT scanning has been evaluated to study RV function. In addition to estimating right ventricular ejection fraction (RVEF), it can estimate RV wall mass. Its use is still experimental, but with further improvement, it may be used to evaluate the progression of cor pulmonale in the near future.
  • Magnetic resonance imaging (MRI) of the heart is another modality that can provide valuable information about RV mass, septal flattening, and ventricular function.5
  • Radionuclide ventriculography can determine RVEF noninvasively. Myocardial perfusion may also show a permanent increase in brightness of the right ventricle.6

Other Tests

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

  • Right axis deviation
  • R/S amplitude ratio in V1 greater than 1 (increase in anteriorly directed forces may be a sign of posterior infarct)
  • R/S amplitude ratio in V6 less than 1
  • P-pulmonale pattern (an increase in P wave amplitude in leads 2, 3, and aVF)
  • S1 Q3 T3 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
  • Increased AP diameter of the chest

Severe RVH may reflect as Q waves in the precordial leads that may be interpreted as anterior myocardial infarction by mistake (on the other hand, since electrical activity of the RV is significantly less than the LV, small changes in RV forces may be lost in ECG).

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.

Procedures

  • Cardiac catheterization: Right-heart catheterization is considered the most precise method for diagnosis and quantification of pulmonary hypertension. It 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 may be for evaluation of the potential reversibility of pulmonary arterial hypertension with vasodilator therapy or when a left heart catheterization is indicated. For related information, see Medscape's Pulmonary Arterial Hypertension Resource Center.
  • Lung biopsy may occasionally be indicated to determine underlying etiology.

More on Cor Pulmonale

Overview: Cor Pulmonale
Differential Diagnoses & Workup: Cor Pulmonale
Treatment & Medication: Cor Pulmonale
Follow-up: Cor Pulmonale
References
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References

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  2. Han MK, McLaughlin VV, Criner GJ, Martinez FJ. Pulmonary Dieases and the heart. Circulation. Dec, 2007;116:2992-3005. [Medline].

  3. Fedullo PF, Auger WR, Kerr KM, Rubin LJ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. Nov 15 2001;345(20):1465-72. [Medline].

  4. Shujaat A, Minkin R, Eden E. Pulmonary hypertension and chronic cor Pulmonale in COPD. Int J Chron Obstruct Pulmon Dis. 2007;2(3):273-82. [Medline].

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  6. Shih WJ, Kousa K, Mitchell B, Huang WS. Permanently increased brightness of Right Ventricle on myocardial perfusion in chronic Cor Pulmonale. J Nucl Cardiol. Mar-Apr 2006;13(2):294-6. [Medline].

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  20. Wiedemann HP, Matthay RA. The management of acute and chronic cor pulmonale. In: Scharf SM, Cassidy SS, eds. Heart-Lung Interactions in Health and Disease. New York, NY: Marcel Dekker; 1989:915.

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Further Reading

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Keywords

cor pulmonale, right heart failure, right ventricular failure, right ventricular hypertrophy, RVH, right ventricular dilatation, pulmonary hypertension, idiopathic primary pulmonary hypertension, cardiopulmonary disease, emphysema, pulmonary thromboembolism, interstitial lung disease, polycythemia vera, sickle cell disease, macroglobulinemia, chronic obstructive pulmonary disease, COPD, chronic bronchitis, pulmonary embolism, pulmonary emboli

exertional dyspnea, syncope with exertion, cor pulmonale, cough, hemoptysis, hoarseness, jaundice, hyperresonance to percussion, diminished breath sounds, wheezing, distant heart sounds, cyanosis, diastolic pulmonary regurgitation murmur, prominent A wave, distended neck veins, right ventricular third heart sound, holosystolic tricuspid insufficiency murmur, pulsatile liver, peripheral edema

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Contributor Information and Disclosures

Author

Ali A Sovari, MD, Research Fellow, Division of Cardiology, David Geffen School of Medicine, University of California, Los Angeles (UCLA)
Ali A Sovari, MD is a member of the following medical societies: American College of Physicians, American Heart Association, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

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

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

Medical Editor

Gregory Joseph Dehmer, MD, Director, Division of Cardiology, Professor, Department of Medicine, Scott & White Clinic, Texas A&M University School of Medicine
Gregory Joseph Dehmer, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, Society for Cardiac Angiography and Interventions, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Eric H Yang, MD, Assistant Professor of Medicine, Director of Coronary Care Unit, University of North Carolina at Chapel Hill School of Medicine
Eric H Yang, MD is a member of the following medical societies: Alpha Omega Alpha
Disclosure: Up to Date Royalty Review panel membership

 
 
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