HEMODYNAMIC DATA AND SURVIVAL IN CHILDREN WITH PULMONARY-HYPERTENSION

Objectives, Using data from a multi-institutional data base, we sought to determine whether hemodynamic data predict duration of survival in children with primary or secondary pulmonary hypertension. Background . Lung transplantation is a therapeutic option for children with pulmo nary hypertension, Appropriate timing of lung transplantation requires reliable methods of predicting duration of survival in potential cand idates, Methods. A regional data base was used to obtain cardiac cathe terization data on 50 children with mean pulmonary artery pressure (mP AP) >25 mm Hg and indexed pulmonary resistance (Rp) >4.5 Wood units, D ata on survival were obtained from the participating centers. Results. There were 15 patients without congenital heart disease (group 1) and 35 patients with congenital heart disease (group 2) for analysis, Act uarial survival at 1, 2 and 5 Sears was 86%, 69% and 69% in group 1 an d 88%, 88% and 77% in group 2, respectively (p = NS), Hemodynamic vari ables that predicted survival on univariate analysis were mean right a trial pressure (mRAP) (p < 0.0001), mPAP (p = 0.034), Rp (p < 0.0001) and pulmonary Bow (p = 0.003), as well as a variable that we generated -mRAP x Rp (p < 0.0001), On multivariate step,vise logistic regression analysis, mRAP x Rp was independently related to survival, A model us ing mRAP x Rp allows for the estimation of probability of death at I a nd 2 years after catheterization. Conclusions. Hemodynamic variables c an predict survival in children with pulmonary hypertension in the pre sence or absence of congenital heart defects, This information can be used to determine the optimal timing of listing for lung transplantati on. (C) 1997 by the American College of Cardiology.