Skip to main content

Main menu

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • For authors
    • Instructions for authors
    • Submit a manuscript
    • Author FAQs
    • Open access
    • COVID-19 submission information
  • Alerts
  • Podcasts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • For authors
    • Instructions for authors
    • Submit a manuscript
    • Author FAQs
    • Open access
    • COVID-19 submission information
  • Alerts
  • Podcasts
  • Subscriptions

A USA-based registry for pulmonary arterial hypertension: 1982–2006

T. Thenappan, S. J. Shah, S. Rich, M. Gomberg-Maitland
European Respiratory Journal 2007 30: 1103-1110; DOI: 10.1183/09031936.00042107
T. Thenappan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
S. J. Shah
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
S. Rich
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
M. Gomberg-Maitland
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

Figures

  • Tables
  • Fig. 1—
    • Download figure
    • Open in new tab
    • Download powerpoint
    Fig. 1—

    Distribution of World Health Organization functional class I pulmonary arterial hypertension patients by age and sex. □: female; ▓: male.

  • Fig. 2—
    • Download figure
    • Open in new tab
    • Download powerpoint
    Fig. 2—

    Kaplan–Meier survival curve for World Health Organization functional class I pulmonary arterial hypertension patients. The number of patients at risk at 0, 5, 10, 15 and 20 yrs was 568, 240, 71, 19 and 3, respectively.

Tables

  • Figures
  • Table 1—

    Clinical and haemodynamic data at the time of diagnosis of pulmonary arterial hypertension

    Clinical dataAll casesIncident casesPrevalent casesp-value#
    Subjects n57882496
    Female7776770.75
    Age yrs48±14 (21–83)51±14 (21–79)48±14 (21–83)0.063
    WHO functional class III–IV8083760.19
    Exercise capacity METs3.5±33.6±1.83.5±2.00.87
    Haemodynamic data
     mRAP mmHg11±710±711±70.037
     mPAP mmHg52±1451±1252±140.30
     PCWP mmHg10±410±410±40.89
    Cardiac index L·min−1·m−22.3±0.92.4±0.82.2±0.90.07
    PA,O2 saturation58±1261±1057±120.015
    PVR Woods unit12.5±7.311±513±70.027
    Acute vasodilator responders4.64.54.60.99
    Aetiology0.12
     Idiopathic/familial483450
     Connective tissue disease304028
     Congenital heart disease111310
     Portal hypertension797
     Anorexigens323
     HIV111
    • Data are presented as %, mean±sd or mean±sd (range), unless otherwise stated. WHO: World Health Organization; METs: metabolic equivalents; mRAP: mean right atrial pressure; mPAP: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PA,O2: alveolar oxygen partial pressure; PVR: pulmonary vascular resistance. #: comparisons are for incident versus prevalent cases of pulmonary arterial hypertension.

  • Table 2—

    Medications at the time of referral

    Medicationn (%)
    Calcium channel blockers182 (32)
    Digoxin102 (18)
    Warfarin164 (28)
    Prostacyclins14 (2)
    Endothelin antagonists18 (3)
    Phosphodiesterase inhibitors5 (1)
    Aspirin77 (13)
    Angiotensin-converting enzyme inhibitors70 (12)
    Angiotensin receptor blockers23 (4)
    β-blockers50 (9)
    Diuretics272 (47)
    Selective serotonin re-uptake inhibitors16 (6)
  • Table 3—

    Baseline clinical characteristics of patients by aetiology of pulmonary arterial hypertension(PAH)

    Clinical DataIPAH/FPAHCTDCongenital heart diseaseAnorexigenHIVPortal hypertensionp-value
    Age yrs45±1455±1542±1252±1140±449±9<0.0001
    Female7587681001370<0.0001
    WHO functional class
     Mean3.0±0.93.2±0.72.8±0.93.1±0.72.7±0.82.9±0.9<0.007
     Class I7310007
     Class II13921194322
     Class III494945564349
     Class IV313924251422
    Exercise capacity METs3.7±3.13.1±2.73.9±2.93.6±3.23.3±3.73.5±3.00.199
    • Data are presented as mean±sd or %, unless otherwise stated. IPAH: idiopathic PAH; FPAH: familial PAH; CTD: connective tissue disease; WHO: World Health Organization; METs: metabolic equivalents.

  • Table 4—

    Haemodynamics at the time of diagnosis for patients in each subtype of pulmonary arterial hypertension(PAH)

    IPAH/FPAHCTDCongenital heart diseaseAnorexigenHIVPortal hypertensionp-value
    mRAP mmHg11±711±610±713±514±512±70.17
    mPAP mmHg56±1348±1154±2252±1250±546±14<0.0001
    PCWP mmHg10±410±413±89±310±212±5<0.0001
    Cardiac index L·min−1·m−22±0.72.3±0.82.8±0.71.9±0.72.3±0.83.2±1.5<0.0001
    PA,O2 saturation57±1157±1166±1454±1354±962±13<0.0001
    PVR Woods unit14±6.811.1±5.913.2±11.413.5±7.110.3±3.37.4±5.3<0.0001
    Acute vasodilator responders5.42.312.50000.12
    • Data are presented as mean±sd or %, unless otherwise stated. IPAH: idiopathic PAH; FPAH: familial PAH; CTD: connective tissue disease; mRAP: mean right atrial pressure; mPAP: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PA,O2: alveolar oxygen partial pressure; PVR: pulmonary vascular resistance.

  • Table 5—

    Comparison of clinical and haemodynamic data for patients diagnosed in three different treatment eras

    Yearp-value
    1982–19961996–20022002–2006
    Subjects n103328147
    Age yr41±1249±1452±14<0.0001
    Female77 (75)253 (77)115 (78)0.81
    WHO functional class3.2±0.92.9±0.83.2±0.80.003
    Exercise capacity METs3.6±3.053.6±3.053.4±2.90.81
    Prostacyclins#3 (2.9)11 (3.4)00.44
    Endothelin antagonists#01 (0.3)17 (11.6)<0.001
    Phosphodiesterase inhibitors#005 (3.4)<0.001
    Idiopathic/familial PAH77 (75)150 (46)49 (33)<0.0001
    CTD12 (11)100 (30)61 (42)<0.0001
    Congenital heart disease10 (10)34 (10)18 (12)0.78
    Portal hypertension4 (4)25 (8)14 (10)0.241
    Anorexigens013 (4)3 (2)0.065
    HIV06 (2)2 (1)0.164
    RAP mmHg12±711±610±60.10
    mPAP mmHg56±1451±1451±120.005
    PCWP mmHg10±410±410±50.40
    Cardiac index L−1·min·m−22.1±0.82.3±0.92.3±0.80.37
    PA,O2 saturation5658600.07
    PVR Wood units13.4±6.412.7±7.911.5±6.10.15
    Acute vasodilator responders3.74.25.80.78
    • Data are presented as mean±sd, % or n (%), unless otherwise stated. WHO: World Health Organization; METs: metabolic equivalents; PAH: pulmonary arterial hypertension; CTD: connective tissue disease; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; PA,O2: alveolar oxygen partial pressure; PVR: pulmonary vascular resistance. #: PAH-specific medications taken by patients at the time of referral to the authors’ centre.

PreviousNext
Back to top
View this article with LENS
Vol 30 Issue 6 Table of Contents
European Respiratory Journal: 30 (6)
  • Table of Contents
  • Index by author
Email

Thank you for your interest in spreading the word on European Respiratory Society .

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
A USA-based registry for pulmonary arterial hypertension: 1982–2006
(Your Name) has sent you a message from European Respiratory Society
(Your Name) thought you would like to see the European Respiratory Society web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Print
Alerts
Sign In to Email Alerts with your Email Address
Citation Tools
A USA-based registry for pulmonary arterial hypertension: 1982–2006
T. Thenappan, S. J. Shah, S. Rich, M. Gomberg-Maitland
European Respiratory Journal Dec 2007, 30 (6) 1103-1110; DOI: 10.1183/09031936.00042107

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero

Share
A USA-based registry for pulmonary arterial hypertension: 1982–2006
T. Thenappan, S. J. Shah, S. Rich, M. Gomberg-Maitland
European Respiratory Journal Dec 2007, 30 (6) 1103-1110; DOI: 10.1183/09031936.00042107
del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo
Full Text (PDF)

Jump To

  • Article
    • Abstract
    • METHODS
    • RESULTS
    • DISCUSSION
    • Acknowledgments
    • References
  • Figures & Data
  • Info & Metrics
  • PDF
  • Tweet Widget
  • Facebook Like
  • Google Plus One

More in this TOC Section

  • Increased hyaluronic acid content in idiopathic pulmonary arterial hypertension
  • The responsiveness and validity of the CAMPHOR Utility Index
  • Inhalation of vasoactive intestinal peptide in pulmonary hypertension
Show more Original Articles: Pulmonary Hypertension

Related Articles

Navigate

  • Home
  • Current issue
  • Archive

About the ERJ

  • Journal information
  • Editorial board
  • Reviewers
  • CME
  • Press
  • Permissions and reprints
  • Advertising

The European Respiratory Society

  • Society home
  • myERS
  • Privacy policy
  • Accessibility

ERS publications

  • European Respiratory Journal
  • ERJ Open Research
  • European Respiratory Review
  • Breathe
  • ERS books online
  • ERS Bookshop

Help

  • Feedback

For authors

  • Instructions for authors
  • Submit a manuscript
  • ERS author centre

For readers

  • Alerts
  • Subjects
  • Podcasts
  • RSS

Subscriptions

  • Accessing the ERS publications

Contact us

European Respiratory Society
442 Glossop Road
Sheffield S10 2PX
United Kingdom
Tel: +44 114 2672860
Email: journals@ersnet.org

ISSN

Print ISSN:  0903-1936
Online ISSN: 1399-3003

Copyright © 2021 by the European Respiratory Society