Pulmonary hypertension natural history
Survival:
The National Institutes of Health (NIH)Registry estimated a median survival of 2.8 years for symptomatic patients with idiopathic pulmonary hypertension who do not recieve any treatment.
1, 3,and 5-year survival rates for untreated patients with idiopathic pulmonary hypertension were 68%, 48%, and 34%, respectively. Studies from Japan,India and Mexico have suggested similar results.
The median survival duration was even lower for patients with pulmonary hypertension that was associated with another disease like portal hypertension, and Scleroderma(2-year survival of 40$ if untreated).
Prognosis
Surprisingly, patients with Eisenmenger syndrome have a more favorable hemodynamic profile and prognosis than adults with idiopathic or primary pulmonary hypertension.[1]
Indicators of Poor Prognosis:
- Age>45 at presentation.
- Poor exercise capacity.
- Elevated Brain Natriuretic Peptide.
- Pericardial effusion.
- Elevated RA size and pressure.
- RV significant dysfunction or failure.
- Low cardiac index.
- Decreased pulmonary arterial capacitance.
- Underlying Scleroderma or Liver disease.
- ↑ Hopkins WE,Ochoa LL, Richardson GW, Trulock EP(1996) Comparison of the hemodynamics and survival or patients with severe pulmonary hypertension or Eisenmenger Syndrome.