Circulation, Vol 89, 1733-1744, Copyright © 1994 by American Heart Association
J Sandoval, O Bauerle, A Palomar, A Gomez, ML Martinez-Guerra, M Beltran and ML Guerrero
BACKGROUND: The prognosis of patients with primary pulmonary hypertension
(PPH) remains a major problem for the planning and assessment of
therapeutic interventions. The objectives of this study were (1) to
characterize mortality in a Mexican population of patients with PPH and to
investigate factors associated with survival and (2) to test the
applicability in this population of the prognostic equation proposed by the
US National Institutes of Health study on PPH. METHODS AND RESULTS: A
dynamic cohort of patients with PPH at our institution were enrolled
between June 1977 and August 1991 and prospectively followed at regular
intervals through September 1992. Measurements at diagnosis included
hemodynamic and pulmonary function variables in addition to information on
demographic data and medical history. The response to vasodilator treatment
was also analyzed. The estimated median survival of the group was 4.04
years (95% confidence interval, 2.98 to 5.08 years). Variables associated
with poor survival (univariate analysis) included an elevated mean right
atrial pressure, a decreased cardiac index, and a decreased mixed venous
PO2. A reduced forced vital capacity and the absence of vasodilator
treatment were also associated with poor survival. A multivariate Cox
proportional- hazards regression analysis was used to assess the adjusted
hazard ratios, hence the relative contributions of the variables
controlling for confounding. Reduced forced vital capacity and cardiac
index and increased right atrial pressure were still significantly
associated as risk factors for survival in patients with PPH. Survival as
computed by the equation correlated with real survival of PPH patients with
positive predictive values of 87%, 91%, and 89% at 1, 2, and 3 years,
respectively. The equation, however, was relatively unable to predict
deaths in our population, in part because of the strict limits of poor
prognosis. CONCLUSIONS: Mortality in PPH is largely associated with
hemodynamic variables that assess right ventricular function. The proposed
prognostic equation had a high sensitivity and a relatively low specificity
to predict survival in our PPH population. To improve this specificity it
may be necessary to increase the limits of poor prognosis as defined by the
equation.
ARTICLES
Survival in primary pulmonary hypertension. Validation of a prognostic equation
Cardiopulmonary Department, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico DF, Mexico.
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