Idiopathic pulmonary arterial hypertension

Last reviewed: 22 Feb 2023
Last updated: 09 Aug 2019

Summary

Definition

History and exam

Key diagnostic factors

  • age 20-50 years
  • female sex
  • dyspnea
  • accentuated pulmonic component (P2) to the second heart sound
  • tricuspid regurgitation murmur
  • family history
More key diagnostic factors

Other diagnostic factors

  • fatigue
  • peripheral edema
  • cyanosis
  • stimulant use
  • syncope
  • chest pain
  • near syncope
  • early diastolic, high-pitched murmur in the pulmonary area
  • jugular vein distension
Other diagnostic factors

Risk factors

  • family history
  • female sex
  • bone morphogenetic protein receptor type 2 (BMPR2) mutations
  • appetite suppressants
  • drugs and toxins
More risk factors

Diagnostic investigations

1st investigations to order

  • chest radiography
  • ECG
  • transthoracic Doppler echocardiography
  • right heart catheterization
  • ANA
  • PFTs
  • nocturnal oximetry
  • ventilation-perfusion (V/Q) scintigraphy
  • 6-minute walk test
  • B-type natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP)
  • CBC
  • LFTs
  • thyroid function tests
  • HIV serology
More 1st investigations to order

Investigations to consider

  • high-resolution chest CT scan ± CT pulmonary angiography
  • vasodilator testing with inhaled nitric oxide, intravenous epoprostenol, or intravenous adenosine
More investigations to consider

Treatment algorithm

ONGOING

positive response to acute vasoreactivity testing with no contraindication to calcium-channel blockers

negative response to acute vasoreactivity testing or calcium-channel blockers contraindicated

Contributors

Authors

Gustavo A. Heresi, MD, MS
Gustavo A. Heresi

Director, Pulmonary Vascular and CTEPH Program

Department of Pulmonary and Critical Care Medicine

Respiratory Institute

Cleveland Clinic

Cleveland

OH

Disclosures

GAH has received speaking and advisory board fees from Bayer Healthcare, the manufacturer of riociguat.

Raed A. Dweik, MD
Raed A. Dweik

Director

Pulmonary Vascular Program

Department of Pulmonary, Allergy, and Critical Care Medicine

Cleveland Clinic

Cleveland

OH

Disclosures

RAD declares that he has no competing interests.

Peer reviewers

Michael J. Krowka, MD

Division of Pulmonary and Critical Care Medicine

Mayo Clinic College of Medicine

Rochester

MN

Disclosures

MJK declares that he has no competing interests.

Charlie Elliot, MB ChB, MRCP

Consultant Physician in Respiratory and General Internal Medicine

Sheffield Pulmonary Vascular Disease Unit

Royal Hallamshire Hospital

Sheffield

UK

Disclosures

CE has received reimbursement for attending several conferences as well as lecture fees from Actelion Pharmaceuticals, GSK, and Bayer.

  • Idiopathic pulmonary arterial hypertension images
  • Differentials

    • Pulmonary arterial hypertension (PAH) associated with left heart diseases (pulmonary venous hypertension)
    • PAH associated with lung respiratory diseases and/or hypoxia
    • PAH due to chronic thrombotic and/or embolic disease
    More Differentials
  • Guidelines

    • Guidelines for the diagnosis and treatment of pulmonary hypertension
    • Pharmacologic therapy for pulmonary arterial hypertension in adults: CHEST guideline and expert panel report
    More Guidelines
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