The term pulmonary hypertension (PH) is applicable to a group of rare conditions which are both chronic and life-threatening. The latest World Health Organization (WHO)classification of five types of PH is from 2008 (Simonneau et al, 2009). These classifications include:

Group 1 covers pulmonary arterial hypertension (PAH) including idiopathic, inherited, drugs or toxins, connective tissue diseases, and congenital heart disease; Group 2 is associated with left-sided heart disease; Group 3 is associated with lung diseases; Group 4 is caused by blood clots in the lungs or blood clotting disorders (CTEPH); and Group 5 is caused by various other disorders associated with multisystemic disease processes (polycythemia, sarcoidosis, metabolic disorders).

Group 1, PAH, accounts for the majority of patients (48.9%) followed by Group 4, CTEPH (19.2%).1 Groups 1 and 4 (referred to herein as PH) are routinely followed up by the specialist centres and prescribed PH-specific drugs. The illness trajectory of patients with underlying cardiac or pulmonary disease (Groups 2 and 3) or multisystemic disease (Group 5) is likely to be substantially different with many of these patients not routinely followed-up by the specialists' centres.1

The main symptoms experienced by people with PH include breathlessness and fatigue, often leading to reduced independence and quality of life.2,3 At best, treatments can reduce symptoms, improve health-related quality of life, improve exercise capacity and extend life expectancy.

Development of emPHasis-10 questionnaire

Development of emPHasis-10 is a collaborative project between the PHA UK and the University of Manchester. Itwas designed to help clinicians assess and improve management of PH by measuring the impact that PH has on a person's life and how this changes over time. The development process, which involved over 250 patients with PH,has been published in the European Respiratory Journal with an accompanying editorial.4,5

Early tests show the emPHasis-10 to be a reliable and valid tool for the assessment of the impact that PH has on patients' daily lives. It is currently being prospectively validated in a longitudinal study which is due for completion in July 2015.

Administering and scoring emPHasis-10

emPHasis-10 consists of 10 items which address breathlessness, fatigue, control and confidence. The questionnaire is short and easy for clinicians to administer in a clinic setting. It is also easy for patients to complete.

It does not require complex analysis or interpretation.Each item is scored on a semantic differential six-point scale (0-5), with contrasting adjectives at each end. A total emPHasis-10 score is derived using simple aggregation of the 10 items. emPHasis-10 scores range from 0 to 50, higher scores indicate worse quality of life.

Comparing consecutive emPHasis-10 scores will provide valuable information for the long-term follow-up of patients.Details regarding ranges of emPHasis-10 score severity and minimal clinically relevant change scores will be available in November 2014.

Using emPHasis-10

The emPHasis-10 can be used for clinical or non-funded research purposes without permission and cost, as long as the integrity of the test is respected. All copyright information must be maintained as they appear on the bottom of emPHasis-10.

For commercial use permission should be sought from PHA UK. It may be appropriate that such use involves a donation to the charity.

Currently emPHasis-10 is available in English only. Versions in other languages are currently under development. Only approved translations of emPHasis-10 which have been through standardized translation procedures should be used to ensure that the validity and measurement properties of the questionnaire are maintained. If you are interested in leading the translation and validation of emPHasis-10 into a different language please contact Dr. Janelle Yorke (Janelle.Yorke@manchester.ac.uk).

Download emPHasis-10 questionnaire
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  1. Health and Social Care Information Centre. National Audit of pulmonary hypertension. 2013; Health and Social Care Information Centre: Part of the Government Statistical Service.
  2. Yorke J,Bundock S, Armstrong I. The imPHact of living with pulmonary hypertension: a qualitative exploration. 2014; Nursing & Health Sciences. In Press
  3. Armstrong I, Harries C, Rochnia N, Yorke J. The trajectory to diagnosis with pulmonary hypertension: a qualitative study. 2012; BMJ Opendoi:10.1136/bmjopen-2011-000806.
  4. Yorke J, Corris P, Gaine S, Gibbs S, Kiely D, Pollock V, Harries C, Armstrong I. emPHasis-10: development of a health-related quality of life measure for pulmonary hypertension. ERJ2014;43, 1106-1113. DOI:10.1183/09031936.00127113
  5. Gomberg-Maitland M, Chen H. Measuring health in pulmonary hypertension: emphasising the right end-point? ERJ 2014; http://ow.ly/tdPDV.