Diagnosis and challenges
How are people with PH diagnosed?
Early and accurate diagnosis is essential as, with earlier diagnosis and appropriate treatment, almost two-thirds of PH patients survive longer than five years. However if not treated, approximately half of PH patients die within two years. Diagnosis is carried out by specialists using a number of methods:
- Echocardiogram
- Electrocardiogram
- Exercise testing (e.g. six-minute walk test)
- Certain blood markers (e.g. cardiac troponin T and/or B-type natriuretic peptide)
However, the only definitive diagnosis requires cardiac catheterization which can provide accurate information about the blood pressure in the heart and pulmonary artery. It is used to confirm the diagnosis of PH and confirm or exclude some of the causes.
Are treatments available?
Currently available pharmacological treatments are only approved to treat one of the five types of PH, pulmonary arterial hypertension (PAH, WHO class l). As a result there is an urgent need for more research to improve understanding of how all types of PH can be diagnosed and treated effectively. There are considerable differences in access to approved treatments and surgery options (e.g. lung transplant) across Europe.
What are the challenges associated with PH today?
- Delayed or inaccurate diagnosis
- Lack of awareness within the expert community as well as the general public
- The few pharmacological treatment options available can only be used to treat PAH, one of the five types of PH and all treatments have significant limitations, so there is a high unmet medical need for more effective therapies
- PH affects every patient differently. An individual approach is difficult to achieve in the limited treatment environment
“More needs to be done to improve the future for people with PH, with greater understanding of the condition and more research into effective treatment options. By raising awareness of PH you can help to share the message that Time Matters to people with PH, giving meaning to the term and enhancing understanding of the true impact of this debilitating, irreversible disease.” Pisana Ferrari, Vice President Pulmonary Hypertension Association (PHA) Europe
References
McLaughlin, VV et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol 2009 28;53(17):1573-619.
Simonneau G et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009;54 (1 Suppl S): S43-54).
Rosenkranz S. Pulmonary hypertension: current diagnosis and treatment. Clin Res Cardiol 2007; 96(8):527–41.
Elliott, C et al. Worldwide physician education and training in pulmonary hypertension: pulmonary vascular disease: the global perspective. CHEST 2010; 137(6):85s-94s.
PuckerUp4PH website http://www.puckerup4ph.com/about-ph.php Accessed January 2010.
Benisty JI. Pulmonary Hypertension. Circulation 2002;106;e192-e194.
Peacock, A. Treatment of Pulmonary Hypertension. BMJ 2003;326;853-836.
Cardiac catheterisation, Pulmonary Hypertension Association (PHA) UK. Available from: http://www.phassociation.uk.com/what_is_ph/cardiac_catheterisation.php . Last accessed: 19 July 2011.
