Pulmonary arterial hypertension (PAH) is a rare and life-threatening disease with a relatively low prevalence in the population – approximately 5-10 cases per one million persons for idiopathic PAH. Hereditary forms constitute a small but important component. PAH also occurs in patients with rheumatic diseases, particularly in connection with systemic sclerosis (about 10-15 percent). This latter group has the worst prognosis, with less than 50 percent three-year survival without modern treatment. Pulmonary arterial hypertension also occurs in other conditions, such as in certain forms of congenital heart disease and as a complication due to the use of certain types of drugs.
Investigation, treatment and follow-up are complex and performed by multidisciplinary teams primarily at university hospitals. Over the past 10 years or so, several very expensive pharmaceuticals have been developed. The pharmaceuticals used for this disease group are often so-called “orphan drugs” with special regard both to the scientific evidence for treatment and to the cost for the patient.
The main purpose is to compile clinical data from diagnosis onward and, through coordination with population and mortality registries, monitor long-term morbidity and mortality. Certain central parameters concerning quality of life, function and biochemistry are registered and pre-specified events are recorded. Through this, we aim to contribute to better quality of care and follow-up for these rare patients.
In the registry, comparisons can be made between specific hospitals and the rest of the country as well as, for certain key statistics, between different centres. New pharmaceuticals and their effectiveness, quality of care and various treatment strategies can be evaluated.
All Swedish PAH centres participate in the registry, with an estimated coverage rate of 90 percent of PAH patients. All reporting is web-based and every reporting unit can produce continuous analyses and reports. Feedback is provided through annual reports for aggregated data and continuous access to the unit’s own data.
Patients with elevated pulmonary circulation pressure according to the accepted definition (see European guidelines). This is classified in five cause groups, of which patients in group 1 (PAH), group 4 (chronic pulmonary embolism) and group 5 are included regardless of treatment. If the patient belongs to group 2 (left-sided heart disease) and group 3 (lung disease), the patient is only included if PAH-specific treatment is given.
The objective of the registry is to include all patients in Sweden with this rare and serious, but in many cases treatable, disease. We have chosen to include all patients in Sweden with the correct diagnosis who were alive on 1 January 2000 or later.
As of the end of 2011, there were 721 patients included, with about 100 new cases per year over the past three years.
We are aiming for virtually full coverage, which is facilitated by the fact that only eight centres in the country investigate and treat these patients.
Note: Variable lists are updated on a regular basis and should therefore be seen as preliminary. For the latest version, please contact the registry.
Category: Circulatory system
Certification level: 3
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