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Idiopathic/heritable pulmonary arterial hypertension
Disease definition
A form of pulmonary arterial hypertension (PAH) characterized by elevated pulmonary arterial resistance leading to right heart failure; it is progressive and potentially fatal. The majority cases have an identifiable genetic cause, but a significant proportion are idiopathic.
ORPHA:422
Classification level: DisorderA summary on this disease is available in Español (2014) Nederlands (2014) Français (2007) Deutsch (2004) Italiano (2004) Slovak (2004, pdf) Polski ()
Detailed information
General public
- Article for general public
- Ukrainian (2021, pdf) - ELF
- Français (2022) - ELF
- Deutsch (2022) - ELF
- Greek (2022) - ELF
- Español (2022) - ELF
- Italiano (2022) - ELF
- Polski (2022) - ELF
- Russian (2022) - ELF
- Svenska (2022) - Socialstyrelsen
- English (2023) - ELF
Guidelines
- Emergency guidelines
- Français (2020, pdf) - Orphanet Urgences
- Clinical practice guidelines
- Français (2020) - PNDS
Disease review articles
- Review article
- English (2013) - Orphanet J Rare Dis
- Clinical genetics review
- English (2020) - GeneReviews
Genetic Testing
- Guidance for genetic testing
- Français (2010, doc) - ANPGM


Additional information
Further information on this disease
Patient-centred resources for this disease
Research activities on this disease
Specialised Social Services
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.