Last reviewed:October 2019
Last updated:August  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

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

Risk factors

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

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
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

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

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 reviewersVIEW 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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