PURPOSE: Advanced lung disease is frequently complicated by interceding pulmonary hypertension (PH). The etiology of this might differ on a disease by disease basis. Similarly, the severity and rate of change of pulmonary artery (PA) pressures might be disease dependent. We sought to compare the prevalence and severity of PH at the time of transplant evaluation and the time of transplant in patients with different forms of advanced lung disease. We also compared the rate of change in PA pressures amongst diseases.
METHODS: All patients transplanted in whom serial measures of PA pressures were available were compared. PH was defined by a mean PAP (mPAP)>25mmHg.
RESULTS: There were 80 patients who qualified for the analysis. Of these, there were 55 with IPF, 14 with COPD, 9 with sarcoidosis and 2 with cystic fibrosis. The baseline prevalence of PH in the IPF group was 40% (22/55), 57% (8/14) in the COPD group and 100% (9/9) in the sarcoidosis group. The baseline mPAP in the IPF group was 23.4mmHg, 24.7mmHg in the COPD group and 37mmHg in the sarcoidosis group. At the time of transplant, prior to implantation, the mPAP of the IPF group was 33.1mmHG with 85% of the patients having PH. At the time of transplantation 64% of the COPD patients had PH with a mPAP for the group of 27mmHg. The mPAP for the sarcoid group at the time of transplant was 37.4mmHg. The rate of change of mPAP for the IPF, COPD and sarcoid patients was 3.15, 0.17 and 0.07mmHg/month, respectively.
CONCLUSION: Sarcoid patients have more significant PH at time of listing for transplantation.The rate of change in PA pressures is significantly greater in patients with IPF than other disease groups.
CLINICAL IMPLICATIONS: The reason for this greater rate of change is uncertain, but might partly explain the generally worse prognosis of IPF patients.
DISCLOSURE: Steven Nathan, None.