Pulmonary hemodynamics and transplant-free survival in sarcoidosis-associated pulmonary hypertension: Results from an international registry.

Pulmonary hypertension (PH) is a risk factor for mortality in patients with sarcoidosis. Severe PH in chronic lung disease has previously been defined as mean pulmonary arterial pressure (mPAP) ≥ 35 mmHg or mPAP 25 ≥ mmHg with cardiac index (CI) ≤ 2 L/min/m ² . However, there is no clear definition denoting severity of sarcoidosis-associated PH (SAPH). We aimed to determine pulmonary hemodynamic cut-off values where transplant-free survival was worse among patients with SAPH. This was a retrospective cohort analysis of the Registry of SAPH database focusing on pulmonary hemodynamic predictors of transplant-free survival among patients with precapillary SAPH. Cox regression was performed to determine which pulmonary hemodynamic values predicted death or lung transplantation. Kaplan-Meier survival analysis was performed on statistically significant predictors to determine pulmonary hemodynamic cut-off values where transplant-free survival was decreased. Decreased transplant-free survival occurred among SAPH patients with mPAP ≥ 40 mmHg and SAPH patients with pulmonary vascular resistance (PVR) ≥ 5 Woods units (WU). Transplant-free survival was not decreased in patients who fulfilled prior criteria of severe PH in chronic lung disease. We identified new cut-offs with decreased transplant-free survival in the SAPH population. Neither cut-off of mPAP ≥ 40 mmHg nor PVR ≥ 5 WU has previously been shown to be associated with decreased transplant-free survival in SAPH. These values could suggest a new definition of severe SAPH. Our PVR findings are in line with the most recent European Society of Cardiology/European Respiratory Society guideline definition of severe PH in chronic lung disease.
Competing Interests: The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: S. J. W. has received honoraria for lectures and advisory boards from Janssen (previously known as Actelion), Bayer, MSD and GSK. He has received travel grants from Janssen and GSK. He has received research grants from Bayer and Janssen. RPB has grant support from Gilead, Bayer, Actelion, Genentech, aTyr, Novartis, and Bellephron for studies in sarcoidosis. O. A. S. consults for and is a speaker for Bayer, United Therapeutics, and Janssen & Janssen. She also consults for Altavant. L. C. P. declares personal fees from Janssen Pharmaceuticals, outside the submitted work. SB has received consultancy fees from aTy and Kinevant. S. D. N. is a consultant for Boehringer‐Ingelheim, Roche, United Therapeutics, Bellerophon, Third Pole, and Merck. He is on the speakers bureau for Boehringer‐Ingelheim and United Therapeutics. R. G. has received consultancy fees from Mallinckrodt. This research did not receive any specific grant from funding agencies in the public, commercial, or not‐for‐profit sectors. The remaining author declare no conflict of interest.
(© 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)