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 pulmonary hypertension. Appropriate timing of lung transplantation requires reliable methods of predicting duration of survival in potential candidates. Methods. A regional data base was used to obtain cardiac catheterization data on 50 children with mean pulmonary artery pressure (mPAP) >25 mm Hg and indexed pulmonary resistance (Rp) >4.5 Wood units. Data 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. Actuarial survival at 1, 2 and 5 years was 86%, 69% and 69% in group 1 and 88%, 88% and 77% in group 2, respectively (p = NS). Hemodynamic variables that predicted survival on univariate analysis were mean right atrial pressure (mRAP) (p < 0.0001), mPAP (p = 0.034), Rp (p < 0.0001) and pulmonary flow (p = 0.003), as well as a variable that we generated-mRAP x Rp (p < 0.0001). On multivariate stepwise logistic regression analysis; mRAP x Rp was independently related to survival. A model using mRAP x Rp allows for the estimation of probability of death at 1 and 2 years after catheterization. Conclusions. Hemodynamic variables can predict survival in children with pulmonary hypertension in the presence or absence of congenital heart defects. This information can be used to determine the optimal timing of listing for lung transplantation.
Bibliographical noteFunding Information:
Dr. Clabby is supported by the American Academy of Pediatrics through the Pediatric Scientist Development Program.