1. Median survival rate in idiopathic PAH -
- 2.8 YEARS
2. % of median survival in IPAH in NIH registry -
- 1 year - 68 %
- 3 year - 48%
- 5 year - 34 %
3 . Indian/ japan studies suggest median survival in IPAH -
- 2-3 years
4. worst prognosis in PAH seen in cases of
- PAH associated with scleroderma
- 2 year survival 40%
5. congenital heart disease with EMS
- prognosis is better than IPAH
- SURVIVAL RATE -
- 1 YEAR -97 %
- 2 YEAR - 89 %
- 3 YEAR - 77 %
6. poor prognosis in PAH indicated by -
- C/F RV failure
- rapid progression of symptoms
- Class IV functional status
- 6 minute walk test < 300 m
- Peak VO2 Max < 10.4 ml / kg/ min
- ECHO -
- pericardial effusion
- RV enlargement
- RA enlargement
- RVdysfunction
- RA pressure > 20 mmHg
- cardiac index < 2.0 l /min/sq. m
- BNP significantly elevated