17 February 2020

National Quality Registry for Pulmonary Arterial Hypertension (PAH)

  • Registry Manager: Region Uppsala
  • Registry Centre - affiliation: Uppsala Clinical Research Center (UCR)
  • Start year: 2007
  • Classifications used: ICD10
  • This registry contains data on: Pharmaceuticals, Diagnoses, Interventions, Inpatient Care, Specialized Outpatient Care, Follow-up Data 3-5 months after the case is registered in the registry, Follow-up Data 6-11 months after the case is registered in the registry, Follow-up Data 12 months or later after the case is registered in the registry

Description of registry

The term Pulmonary Hypertension (PH) is synonymous with high blood pressure in the lung circulation. The most common causes leading to PH are diseases related to the left side of the heart and diseases affecting the lung parenchyma and/or the airways. Much more seldom, PH may be caused by structural changes of the lung vessels in which case the term Pulmonary Arterial Hypertension (PAH) is used.

PAH may appear spontaneously and without an apparent cause (idiopathic PAH) or in association with other diseases (associated PAH). The most common causes of associated PAH are connective tissue disease and congenital heart disease with septal defect/shunt. Acute pulmonary embolism may, in rare cases, lead to high pressure in the lung circulation and a PAH-like disease, known as CTEPH.

The most common symptoms of pulmonary hypertension are shortness of breath in connection with physical activity and general tiredness. These are, however, common symptoms, which most often - after routine examination with chest x-ray, ECG, ultrasound of the heart, and pulmonary functional tests - are shown to be caused by left heart disease or lung disease. If, after the standard investigation, the suspicion of PAH or CTEPH remains, the patients should be further evaluated by right heart catheterization for accurate measurements of the blood pressure and flow in the lung circulation.

At present, several medical and surgical therapeutic alternatives for patients with PAH and CTEPH are available, all of which may improve quality of life and survival. Nevertheless, PAH and CTEPH are still serious conditions and early diagnosis and treatment may be critical for the patients wellbeing and outcome.

Patients suspected of having PAH or CTEPH require a careful evaluation and complex diagnostic procedures, as well as, systematic follow-ups in case the diagnosis is confirmed; therefore the medical care of these patients should be handled by expert PAH/CTEPH centres.

Patient data from diagnosis and from follow-up visits are reported to the Swedish PAH Register (SPAHR). Thus, the register is a useful instrument for analysis of short- and long-time outcomes, contributing to improved quality of care, treatment, and prognosis of patients across the country.

The register offers, at the same time, an appropriate platform for evaluation of new treatment strategies, including new medicines and surgical procedures.

Population

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.

Ages in the registry

All ages.

Total volume of registry

As of the end of 2019, there were about 1 600 patients included, with about 100 new cases per year.

Coverage rate

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: 2

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