Secondary right heart disease


pulmonary arterial hypertension: Pulmonary arterial hypertension (PAH) is a group of diseases characterized by elevated pulmonary arterial resistance leading to right heart failure. PAH is progressive and potentially fatal. PAH may be idiopathic and/ or familial, or induced by drug or toxin (drug-or toxin-induced PAH, see these terms) or associated with other diseases like congenital heart disease, connective tissue disease, HIV, schistosomiasis, portal hypertension (PAH associated with other disease, see this term).

Data source
FinnGen phenocode I9_SECONDRIGHT
Hospital Discharge registry ICD-10: I27, ICD-9: 416, ICD-8: 426
Cause of Death registry ICD-10: I27, ICD-9: 416, ICD-8: 426
Level in the ICD-hierarchy 4
First defined in version DF3
DOID 6432
GWAS catalog 0001361
SNOMED CT 11399002

Key figures

Sex All Female Male
Number of individuals 246 140 106
Unadjusted prevalence (%) 0.19 0.19 0.18
Mean age at first event (years) 65.66 63.98 67.86
Median number of events / individual 1 2 1

Clinical metrics

Sex All Female Male
Recurrence within 6 months (%) 42.28 47.14 35.85
Case fatality at 5-years (%) 30.89 25.0 38.68