eMedicine Specialties > Pulmonology > Pulmonary Hypertension

Pulmonary Hypertension, Secondary: Differential Diagnoses & Workup

Author: Nader Kamangar, MD, FACP, FCCP, FAASM,, Associate Professor of Clinical Medicine, Director of Hospitalist/Intensivist Program, Division of Pulmonary, Critical Care and Sleep Medicine, David Geffen School of Medicine at University of California Los Angeles; Associate Director, Combined Pulmonary and Critical Care Fellowship Program, Cedars-Sinai/Olive View-UCLA/West Los Angeles Veterans Affairs Medical Center
Coauthor(s): Shahriar Pirouz, MD, Resident Physician, Department of Internal Medicine, Olive View University of California Los Angeles Medical Center; Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital
Contributor Information and Disclosures

Updated: Aug 21, 2009

Differential Diagnoses

Apnea, Sleep
Emphysema
Arteriovenous Malformations
Mitral Regurgitation
Atrial Myxoma
Mitral Stenosis
Atrial Septal Defect
Pulmonary Hypertension, Primary
Cardiomyopathy, Dilated
Restrictive Lung Disease
Cardiomyopathy, Hypertrophic
Systemic Lupus Erythematosus
Cardiomyopathy, Restrictive
Chronic Obstructive Pulmonary Disease

Workup

Laboratory Studies

Arterial blood gas determinations should be performed in pulmonary hypertension (PH) patients to assess for hypoxemia.  

  • A collagen-vascular disease screening should be performed. This includes measuring the erythrocyte sedimentation rate, rheumatoid factor levels, and antinuclear antibody levels.
  • Synthetic liver function test results (ie, albumin levels, prothrombin time, bilirubin levels) may indicate liver disease associated with portal hypertension.
  • HIV testing and hepatology serology tests should be performed on patients at risk.
  • A complete blood cell count, biochemistry panel, prothrombin time, and activated partial thromboplastin time should be performed at baseline. 

Imaging Studies

  • Chest radiography
    • The classic finding on a chest radiograph from a patient with pulmonary arterial hypertension is enlargement of central pulmonary arteries, attenuation of peripheral vessels, and oligemic lung fields.
    • Findings of right ventricular and right atrial dilatation are possible.


Chest radiograph of a patient with secondary pulm...

Chest radiograph of a patient with secondary pulmonary hypertension shows enlarged pulmonary arteries. This patient had an atrial septal defect.

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Chest radiograph of a patient with secondary pulm...

Chest radiograph of a patient with secondary pulmonary hypertension shows enlarged pulmonary arteries. This patient had an atrial septal defect.



A 54-year-old woman with history of scleroderma (...

A 54-year-old woman with history of scleroderma (CREST variety, ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened upon exertion. Images from a high-resolution CT scan of the lungs showed no parenchymal disease. The patient was found to have severe pulmonary arterial hypertension.

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A 54-year-old woman with history of scleroderma (...

A 54-year-old woman with history of scleroderma (CREST variety, ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened upon exertion. Images from a high-resolution CT scan of the lungs showed no parenchymal disease. The patient was found to have severe pulmonary arterial hypertension.

  • Two-dimensional echocardiography
    • Signs of chronic right ventricular pressure overload are present, which include increased thickness of the right ventricle with paradoxical bulging of the septum into the left ventricle during systole.
    • In later stages, right ventricular dilatation occurs, leading to right ventricular hypokinesis.
    • Right atrial dilatation and tricuspid regurgitation are also present.
  • Doppler echocardiography
    • Doppler echocardiography is the most reliable noninvasive method to estimate pulmonary arterial pressure.
    • Tricuspid regurgitation is usually present in patients with pulmonary arterial hypertension, which aids measurement of pulmonary artery pressure when using the modified Bernoulli equation. The efficacy of Doppler echocardiography depends on the ability to adequately locate the tricuspid regurgitant jet. Furthermore, acoustic windows may be limited in patients who have other diseases (eg, chronic obstructive pulmonary disease [COPD]) or in those who are obese.
    • Tricuspid regurgitation is generally detected in more than 90% of patients with severe SPAH, and a correlation of greater than 95% is observed when the pressure is measured using catheterization. Doppler echocardiography is a useful noninvasive test for long-term follow-up.

Other Tests

  • Electrocardiography
    • Signs of right ventricular hypertrophy or strain may be observed.
    • These include right axis deviation, an R-to-S wave ratio greater than 1 in lead V1, increased P-wave amplitude, and an incomplete or complete right bundle-branch block pattern.
  • Ventilation perfusion lung scanning
    • Ventilation perfusion scan should be performed to exclude chronic thromboembolic pulmonary hypertension. A high- or low-probability scan result is most useful, whereas intermediate-probability results should lead to performing pulmonary angiography.
    • Diffuse mottled perfusion can be observed in patients with primary pulmonary hypertension, as opposed to segmental or subsegmental mismatched defects observed in patients with SPAH.


A ventilation/perfusion scan of bilateral mismatc...

A ventilation/perfusion scan of bilateral mismatched segmental and subsegmental defects, suggesting chronic thromboembolic hypertension.

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A ventilation/perfusion scan of bilateral mismatc...

A ventilation/perfusion scan of bilateral mismatched segmental and subsegmental defects, suggesting chronic thromboembolic hypertension.

  • Pulmonary function testing
    • Pulmonary function tests (ie, spirometry and diffusing capacity for carbon monoxide) should be performed in patients with SPAH to exclude an underlying pulmonary disorder. Diffusing capacity is universally reduced in patients with pulmonary hypertension.
    • These tests may show an obstructive pattern suggestive of COPD or a restrictive pattern suggestive of an interstitial lung disease. Furthermore, the severity of the lung disorder may be established by pulmonary function test findings because they provide both the qualitative and quantitative data.

Procedures

  • Right-sided heart catheterization
    • Right-sided heart catheterization is the criterion standard test for the diagnosis, quantification, and characterization of pulmonary arterial hypertension. Left-sided heart dysfunction and intracardiac shunts can be excluded, and the cardiac output can be measured.
    • The indications for this procedure are (1) difficulty with the accurate measurement of pulmonary arterial hypertension with Doppler echocardiography and (2) the need for a precise measurement of pulmonary vascular resistance to conduct a vasodilator trial to assess the acute response to vasodilators.
    • Acute vasoreactivity is determined by administering a short-acting vasodilator such as prostacyclin, inhaled nitric oxide, or adenosine. An acute response often predicts a beneficial effect from oral agents, such as calcium channel blockers.12


This left pulmonary arterial angiogram shows larg...

This left pulmonary arterial angiogram shows large central pulmonary arteries and attenuation of peripheral vessels, but thrombosis cannot be identified because it has organized along the vessel walls.

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This left pulmonary arterial angiogram shows larg...

This left pulmonary arterial angiogram shows large central pulmonary arteries and attenuation of peripheral vessels, but thrombosis cannot be identified because it has organized along the vessel walls.



Bilateral angiogram should be performed in patien...

Bilateral angiogram should be performed in patients suggested to have chronic thromboembolic pulmonary arterial hypertension. This right pulmonary arterial angiogram from the patient in Media File 8 again shows no evidence of a filling defect, therefore excluding acute thrombosis. Angioscopy is a potentially useful procedure in this setting.

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Bilateral angiogram should be performed in patien...

Bilateral angiogram should be performed in patients suggested to have chronic thromboembolic pulmonary arterial hypertension. This right pulmonary arterial angiogram from the patient in Media File 8 again shows no evidence of a filling defect, therefore excluding acute thrombosis. Angioscopy is a potentially useful procedure in this setting.

Histologic Findings

The histopathologic lesions in patients with secondary pulmonary hypertension are similar to those observed in patients with primary pulmonary hypertension. These pathological changes are the result of long-standing hypertension rather than a consequence of different causes.

The plexiform lesion is observed in patients with all types of pulmonary arterial hypertension. These lesions consist of medial hypertrophy, eccentric or concentric laminar intimal proliferation and fibrosis, fibrinoid degeneration, and thrombotic lesions. Fresh or organized and recanalized thrombi may also be present. Diverse types of intimal and muscular lesions of the small muscular arteries may cause the clinical syndrome of pulmonary hypertension, and a plexiform lesion reflecting the abrupt onset of pulmonary hypertension is likely, rather than the lesion being a distinctive cause.

More on Pulmonary Hypertension, Secondary

Overview: Pulmonary Hypertension, Secondary
Differential Diagnoses & Workup: Pulmonary Hypertension, Secondary
Treatment & Medication: Pulmonary Hypertension, Secondary
Follow-up: Pulmonary Hypertension, Secondary
Multimedia: Pulmonary Hypertension, Secondary
References
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Further Reading

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Contributor Information and Disclosures

Author

Nader Kamangar, MD, FACP, FCCP, FAASM,, Associate Professor of Clinical Medicine, Director of Hospitalist/Intensivist Program, Division of Pulmonary, Critical Care and Sleep Medicine, David Geffen School of Medicine at University of California Los Angeles; Associate Director, Combined Pulmonary and Critical Care Fellowship Program, Cedars-Sinai/Olive View-UCLA/West Los Angeles Veterans Affairs Medical Center
Nader Kamangar, MD, FACP, FCCP, FAASM, is a member of the following medical societies: American Academy of Sleep Medicine, American Association of Bronchology, American College of Chest Physicians, American College of Physicians, American Lung Association, American Medical Association, American Thoracic Society, California Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Shahriar Pirouz, MD, Resident Physician, Department of Internal Medicine, Olive View University of California Los Angeles Medical Center
Disclosure: Nothing to disclose.

Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital
Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Oleh Wasyl Hnatiuk, MD, Program Director, National Capital Consortium, Pulmonary and Critical Care, Walter Reed Army Medical Center; Associate Professor, Department of Medicine, Uniformed Services University of Health Sciences
Oleh Wasyl Hnatiuk, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

,, Kathy Roarty Placeholder
Disclosure: Nothing to disclose.

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

 
 
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