Pulmonary hypertension natural history

Assistant Editor(s)-in-Chief: Ralph Matar,

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, with the cause of death usually being right ventricular failure (cor pulmonale).


 * 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 other diseases like portal hypertension, and Scleroderma(2-year survival of 40% if untreated).


 * A recent outcome study of those patients who had started treatment with bosentan (Tracleer®) showed that 86% patients were alive at 3 years.


 * With multiple agents now available, combination therapy is increasingly used. Impact of these agents on survival is not known, since many of them have been developed only recently.


 * It would not be unreasonable to expect median survival to extend past 10 years in the near future.

Prognosis

 * The long-term prognosis has been known to be poor, however outcomes have changed dramatically over the last two decades. This may be attributed to the use of newer drug therapy, better overall care, and earlier diagnosis (lead time bias).


 * Some people with this condition may have heart failure that could lead to death. Assessment of prognosis in patients with pulmonary arterial hypertension (PAH) is important since it influences both medical therapy and referral for transplantation.

Surprisingly, patients with Eisenmenger syndrome have a more favorable hemodynamic profile and prognosis than adults with idiopathic or primary pulmonary hypertension.

Indicators of Poor Prognosis:
 * Age>45 at presentation.
 * Poor exercise capacity.
 * Elevated Brain Natriuretic Peptide.
 * Pericardial effusion.
 * Elevated right atrial size and pressure.
 * Significant right ventricular dysfunction or failure.
 * Low cardiac index.
 * Decreased pulmonary arterial capacitance.
 * Underlying Scleroderma or liver disease.