Last reviewed: November 2017
Last updated: November  2017

Summary

Definition

History and exam

Key diagnostic factors

  • age 20 to 50 years
  • female gender
  • FHx
  • dyspnea
  • accentuated pulmonic component (P2) to the second heart sound
  • tricuspid regurgitation murmur

Other diagnostic factors

  • peripheral edema
  • cyanosis
  • stimulant use
  • fatigue
  • syncope
  • chest pain
  • near syncope
  • palpitations
  • leg edema
  • right-sided third heart sound (S3)
  • right-sided fourth heart sound (S4)
  • early diastolic, high-pitched murmur in the pulmonary area
  • palpable left parasternal heave
  • jugular vein distention

Risk factors

  • FHx
  • female gender
  • bone morphogenetic protein receptor type 2 (BMPR2) mutations
  • drugs and toxins

Diagnostic investigations

1st investigations to order

  • chest radiography
  • ECG
  • transthoracic Doppler echocardiography
  • right heart catheterization
  • ANA
  • PFTs
  • nocturnal oximetry
  • ventilation-perfusion scintigraphy
  • 6-minute walk test
  • B-type natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP)
  • CBC
  • LFTs
  • TFTs
  • HIV serology
Full details

Investigations to consider

  • high-resolution chest CT scan ± CT pulmonary angiography
  • vasodilator testing with inhaled nitric oxide, intravenous epoprostenol, or intravenous adenosine
Full details

Treatment algorithm

Contributors

Authors VIEW ALL

Gustavo A. Heresi

Medical Director, Pulmonary Thromboendarterectomy 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

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 VIEW ALL

Division of Pulmonary and Critical Care Medicine

Mayo Clinic College of Medicine

Rochester

MN

Disclosures

MJK declares that he has no competing interests.

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.

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