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Secondary Pulmonary Hypertension Clinical Presentation

  • Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Ryland P Byrd, Jr, MD  more...
 
Updated: Jan 27, 2016
 

History

The clinical manifestations of secondary pulmonary arterial hypertension (SPAH) are frequently masked by the underlying disease entities. Obtaining a careful history may help exclude some of the numerous causes of SPAH. Important clues to a specific secondary cause include the following:

  • Past history of heart murmur
  • Deep venous thrombosis (DVT) or pulmonary embolism (PE)
  • Raynaud phenomenon
  • Arthritis or arthralgias
  • Rash
  • Heavy alcohol consumption
  • Hepatitis
  • Heavy snoring
  • Daytime hypersomnolence
  • Morning headaches
  • Morbid obesity
  • Family history of pulmonary hypertension
  • Drug use, in particularly diet drugs
  • Medications

Patients with SPAH often have nonspecific symptoms that reflect the underlying etiology. Other symptoms include the following:

  • Dyspnea upon exertion
  • Fatigue
  • Lethargy
  • Syncope with exertion
  • Chest pain
  • Anorexia
  • Right upper quadrant pain

Less common symptoms include the following:

  • Cough
  • Hemoptysis
  • Hoarseness (due to compression of the recurrent laryngeal nerve by the distended pulmonary artery)

Typical exertional angina has been reported in as many as 8.5% of patients with SPAH secondary to mitral stenosis. This most likely occurs because of pulmonary artery distention, right ventricular ischemia, or both.

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Physical Examination

Physical examination findings may include the following:

  • The intensity of the pulmonic component of the second heart sound (P 2) may be increased and the P 2 may demonstrate fixed or paradoxic splitting. A systolic ejection murmur may be heard over the left sternal border. The murmur may be augmented by inspiration. A right ventricular heave may be palpated.
  • A prominent A wave may be observed in the jugular venous pulse. A right-sided fourth heart sound (S 4) with a left parasternal heave may be auscultated.
  • Right ventricular failure leads to systemic venous hypertension and cor pulmonale. The signs of right ventricular failure include a high-pitched systolic murmur of tricuspid regurgitation, hepatomegaly, a pulsatile liver, ascites, and peripheral edema. In this scenario, a right ventricular third heart (S 3) sound is also heard.
  • Signs of underlying cardiac, pulmonary, hepatic, or collagen-vascular disease are often present.

Patients with SPAH often develop cor pulmonale, which further worsens hypoxemia and perpetuates pulmonary hypertension.

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Contributor Information and Disclosures
Author

Nader Kamangar, MD, FACP, FCCP, FCCM Professor of Clinical Medicine, University of California, Los Angeles, David Geffen School of Medicine; Chief, Division of Pulmonary and Critical Care Medicine, Vice-Chair, Department of Medicine, Olive View-UCLA Medical Center

Nader Kamangar, MD, FACP, FCCP, FCCM is a member of the following medical societies: Academy of Persian Physicians, American Academy of Sleep Medicine, American Association for Bronchology and Interventional Pulmonology, American College of Chest Physicians, American College of Critical Care Medicine, American College of Physicians, American Lung Association, American Medical Association, American Thoracic Society, Association of Pulmonary and Critical Care Medicine Program Directors, Association of Specialty Professors, California Sleep Society, California Thoracic Society, Clerkship Directors in Internal Medicine, Society of Critical Care Medicine, Trudeau Society of Los Angeles, World Association for Bronchology and Interventional Pulmonology

Disclosure: Nothing to disclose.

Coauthor(s)

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, World Medical Association

Disclosure: Nothing to disclose.

Kelvin Chan, MD Resident Physician, Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, Olive View-UCLA Medical Center

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Ryland P Byrd, Jr, MD Professor of Medicine, Division of Pulmonary Disease and Critical Care Medicine, James H Quillen College of Medicine, East Tennessee State University

Ryland P Byrd, Jr, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Acknowledgements

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.

Shahriar Pirouz, MD Resident Physician, Department of Internal Medicine, Olive View-UCLA Medical Center

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

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Gross pathology on patient who died of severe pulmonary arterial hypertension secondary to persistent patent ductus arteriosus.
Close-up view of gross pathology on patient who died of severe arterial pulmonary hypertension secondary to persistent patent ductus arteriosus.
During pulmonary arterial thromboendarterectomy, bilateral proximal thrombus was carefully dissected and extracted, leading to resolution of secondary pulmonary arterial hypertension.
Chest radiograph of patient with secondary pulmonary arterial hypertension shows enlarged pulmonary arteries. This patient had atrial septal defect.
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. The patient was found to have severe pulmonary arterial hypertension.
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 on exertion. Spiral CT showed enlarged pulmonary arteries but no evidence of thromboembolism.
Ventilation-perfusion scan of bilateral mismatched segmental and subsegmental defects, suggesting chronic thromboembolic hypertension.
Left pulmonary arterial angiogram shows large central pulmonary arteries and attenuation of peripheral vessels, but thrombosis cannot be identified, because it has organized along vessel walls.
Bilateral angiography should be performed in patients suspected of having chronic thromboembolic pulmonary arterial hypertension. This right pulmonary arterial angiogram shows no evidence of filling defect, therefore excluding acute thrombosis. Angioscopy is potentially useful in this setting.
 
 
 
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