Send to:

Choose Destination
See comment in PubMed Commons below
J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D42-50. doi: 10.1016/j.jacc.2013.10.032.

Definitions and diagnosis of pulmonary hypertension.

Author information

  • 1Department of Respiratory Medicine and German Center for Lung Research, Hannover Medical School, Hannover, Germany. Electronic address:
  • 2Department of Pulmonary Medicine, VU University Medical Center, Amsterdam, the Netherlands.
  • 3Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom.
  • 4College of Medicine, Mayo Clinic, Rochester, Minnesota.
  • 5University of Michigan Scleroderma Program, Ann Arbor, Michigan.
  • 6Department of Pulmonary Circulation and Thromboembolic Diseases, Medical Centre of Postgraduate Medication, Warsaw, Poland.
  • 7Center for Pulmonary Vascular Disease, Division of Cardiology, Jewish General Hospital, McGill University, Montreal, Quebec, Canada.
  • 8Department of Experimental, Diagnostic and Specialty Medicine-DIMES, Bologna University Hospital, Bologna, Italy.
  • 9Division of Cardiology, Kyorin University School of Medicine, Tokyo, Japan.
  • 10Pulmonary Hypertension Program, University of Texas Southwestern Medical Center, Dallas, Texas.
  • 11Experimental Medicine, Imperial College London, London, United Kingdom.
  • 12Division of Pulmonary Sciences and Critical Care Medicine and Cardiology, University of Colorado, Denver, Colorado.


Pulmonary hypertension (PH) is defined by a mean pulmonary artery pressure ≥ 25 mm Hg at rest, measured during right heart catheterization. There is still insufficient evidence to add an exercise criterion to this definition. The term pulmonary arterial hypertension (PAH) describes a subpopulation of patients with PH characterized hemodynamically by the presence of pre-capillary PH including an end-expiratory pulmonary artery wedge pressure (PAWP) ≤ 15 mm Hg and a pulmonary vascular resistance >3 Wood units. Right heart catheterization remains essential for a diagnosis of PH or PAH. This procedure requires further standardization, including uniformity of the pressure transducer zero level at the midthoracic line, which is at the level of the left atrium. One of the most common problems in the diagnostic workup of patients with PH is the distinction between PAH and PH due to left heart failure with preserved ejection fraction (HFpEF). A normal PAWP does not rule out the presence of HFpEF. Volume or exercise challenge during right heart catheterization may be useful to unmask the presence of left heart disease, but both tools require further evaluation before their use in general practice can be recommended. Early diagnosis of PAH remains difficult, and screening programs in asymptomatic patients are feasible only in high-risk populations, particularly in patients with systemic sclerosis, for whom recent data suggest that a combination of clinical assessment and pulmonary function testing including diffusion capacity for carbon monoxide, biomarkers, and echocardiography has a higher predictive value than echocardiography alone.

Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.


CO; CTD; DLCO; HFpEF; HPAH; IPAH; LVEDP; N-terminal pro–B-type natriuretic peptide; NT-proBNP; PAH; PAPm; PAWP; PH; PVR; RHC; SSc; WU; Wood units; cardiac output; connective tissue disease; diagnosis; diffusion capacity for carbon monoxide; heart failure with preserved ejection fraction; hemodynamics; heritable pulmonary arterial hypertension; idiopathic pulmonary arterial hypertension; left ventricular end-diastolic pressure; mean pulmonary artery pressure; pulmonary arterial hypertension; pulmonary artery wedge pressure; pulmonary hypertension; pulmonary vascular resistance; right heart catheterization; scleroderma; screening

Republished in

[PubMed - indexed for MEDLINE]
Free full text
PubMed Commons home

PubMed Commons

How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science
    Loading ...
    Write to the Help Desk