Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension

Primary pulmonary hypertension (PPH) is a fatal illness. In advanced stages only transplantation is able to increase survival. Echocardiography is useful for the assessment of these patients, but there is limited information about its prognostic value. With this goal, 25 consecutive patients, age: 36.7 12.7 years, were studied and followed up for a mean period of 29 months (range: 0.2-84). Eleven echocardiographic parameters of cardiac anatomy, function, and hemodynamics were assessed. Age and sex were also analyzed. Death and heart-lung transplantation were considered end-points. Thirteen events (Death: 8; transplantation: 5) occurred in the follow-up (11 of 13 in the first year). Kaplan-Meier estimated survival free from transplantation at 5 years was 40% (95% CI: 23%-70%). In the univariate analysis, RAA (HR: 1.1, P .0004), TR (HR: 2.7, P .02), and RVET (HR: 0.98, P .02) showed statistically significant relation with survival free from transplantation. Multivariate analysis showed that RAS (HR: 1.10, 95% CI: 1.04-1.17, P .001) and TR (HR: 2.52, 95% CI: 1.01-6.3, P .047) were independent risk factors of transplantation and death. The use of these findings on the management of patients with PPH should be tested in larger studies. (J Am Soc Echocardiogr 2002;15:1160-4.)