Most patients with Primary Pulmonary Hypertension (PPH) have severe exertional limitation which ultimately leads to right heart failure and death. The purpose of the study was to assess the correlation between right ventricular (RV) systolic and diastolic noninvasive variables and exercise tolerance, as well as the predictors of adverse outcome in treated patients.
Methods: We prospectively studied 29 patients, 17 with PPH and 12 with PPH due to collagen disease. RV parameters were assessed by echocardiography and Radionuclide ventriculography. Pulmonary function and clinical profile were assessed by 6 min walk test and NYHA class. The patients were followed-up during 2 years for cardiac death and cardiac deterioration.
Results: Mean age was 51 +/- 15 years, 22 (78%) women. NYHA class1 in 2 pts, class 2 in 17, class 3 in 8 and class 4 in 2 pts. Pulmonary function (DLCO) was low in 25 (86%) pts, mean 22 +/- 48%. Six minutes walk distance was 358 +/- 132 m, RVEF was 34 +/- 11% (range 16-51%). Among RV variables, RVEF, RA area and TR were independently correlated to 6 min walk. Within follow up of 2 years, there were 10 patients with adverse outcome (4 deaths and 6 deteriorated to NYHA class 3 and 4). Among all clinical and noninvasive variables, RVEF only was correlated to adverse outcome.
Conclusion: The noninvasive tests of RVEF, RA size and TR were closely correlated to exercise tolerance. However, among the various clinical, functional and RV variables, RVEF was the only variable correlated with adverse outcome in pts with PPH.