Primary Pulmonary Hypertension Clinical Presentation

  • Author: Ronald J Oudiz, MD, FACP, FACC, FCCP; Chief Editor: Zab Mosenifar, MD   more...
 
Updated: May 16, 2012
 

History

The average time from symptom onset to diagnosis has been reported to be approximately 2 years. Despite recent attempts at increasing the awareness of pulmonary arterial hypertension (PAH), especially associated PAH (APAH), this delay in diagnosis has not changed appreciably in recent years.

Early symptoms are nonspecific. Often, neither the patient nor the physician recognizes the presence of the disease, which leads to delays in diagnosis. Complicating matters, idiopathic PAH (IPAH) requires an extensive workup in an attempt to elucidate an identifiable cause of the elevated pulmonary artery pressure.

The most common symptoms and their frequency, reported in a national prospective study, are as follows:

  • Dyspnea (60% of patients)
  • Weakness (19%)
  • Recurrent syncope (13%)

Women are more likely to be symptomatic than men.

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Physical Examination

Physical findings in persons with PAH can be quite variable.

Cardiovascular examination often reveals the following findings:

  • The pulmonic component of the second heart sound is usually increased, which may demonstrate fixed or paradoxic splitting in the presence of severe right ventricular dysfunction; occasionally, the second heart sound may be palpable.
  • Pulmonic regurgitation (Graham Steell murmur) may be apparent.
  • A murmur of tricuspid regurgitation can be present, and a right ventricular lift (heave) may be noted.
  • Jugular venous pulsations may be elevated in the presence of volume overload, right ventricular failure, or both; large V waves are often present because of the commonly present severe tricuspid regurgitation.

Other findings may include hepatomegaly with palpable pulsations of the liver and an abnormal abdominal-jugular reflex. In untreated patients and patients with worsening decompensated right heart failure, ascites is not uncommonly present.

Lung examination findings are usually normal.

Extremity examination may reveal pitting edema of varying degrees. Patients who are bedridden may have presacral edema.

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Complications

Complications of IPAH include the following:

  • Advanced right-sided heart failure with hepatic congestion
  • Pedal edema
  • Pleural effusions
  • Ascites
  • Worsening dyspnea upon exertion
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Contributor Information and Disclosures
Author

Ronald J Oudiz, MD, FACP, FACC, FCCP  Professor of Medicine, University of California, Los Angeles, David Geffen School of Medicine; Director, Liu Center for Pulmonary Hypertension, Division of Cardiology, LA Biomedical Research Institute at Harbor-UCLA Medical Center

Ronald J Oudiz, MD, FACP, FACC, FCCP is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Heart Association, and American Thoracic Society

Disclosure: Actelion Grant/research funds Clinical Trials + honoraria; Encysive Grant/research funds Clinical Trials + honoraria; Gilead Grant/research funds Clinical Trials + honoraria; Pfizer Grant/research funds Clinical Trials + honoraria; United Therapeutics Grant/research funds Clinical Trials + honoraria; Lilly Grant/research funds Clinical Trials + honoraria; LungRx Clinical Trials + honoraria; Bayer Grant/research funds Consulting; Medtronic Consulting fee Consulting; Novartis Consulting fee Consulting

Specialty Editor Board

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.

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

Disclosure: Medscape Salary Employment

Chief Editor

Zab Mosenifar, MD  Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Professor and Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center, University of California, Los Angeles, David Geffen School of Medicine

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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CADD Legacy ambulatory infusion pump. Courtesy SIMS Deltec, St. Paul, Minn.
Two-dimensional short-axis echocardiogram image. Note the flattened interventricular septum due to right ventricular overload.
 
 
 
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