Pulmonary Arterial Hypertension

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

7.