This issue of Advances in Pulmonary Hypertension focuses on forms of pulmonary hypertension (PH) for which Food and Drug Administration–approved specific therapies are limited or nonexistent. While these diseases share some similarities in clinical presentation and hemodynamics with pulmonary arterial hypertension (PAH), there are differences in pathogenesis and, often, the coexistence of lung or left-sided heart disease which make clinical management more challenging. We are thankful to our authors and roundtable participants for their outstanding contributions to this issue. A review of these articles will demonstrate that these diseases, while similarly affecting the pulmonary vasculature, are quite different from each other. In reading through this issue, we are impressed with how clinically useful these papers will be for all clinicians involved in the care of people living with PH.
Mazen O. Al-Qadi and H. James (Jimmy) Ford start off the issue by providing an updated review of the complexity of sarcoidosis-associated PH. We understand that, in individual patients, the contributions of pulmonary arteriopathy, left-sided cardiac dysfunction, interstitial lung disease, and chronic thromboembolism to sarcoidosis-associated PH is variable, and this has impact on the therapeutic approach. Their review is a helpful, focused overview for clinicians at all levels of clinical experience, and they discuss how to approach off-label use of PAH therapy in segments of this population.
We really enjoyed the overview of the rare and extremely challenging pulmonary veno-occlusive disease (PVOD) written by Marc Humbert and David Montani. This is one of the most comprehensive overviews of the biology, diagnostic approach, and management of PVOD we have read. It is a must for all involved in the care of patients with PH, as PVOD is a critical diagnosis not to be missed, especially as many of our therapies are not well tolerated in these patients. This paper is coupled with the report by Martin Rofael et al. on the use of lung transplantation in patients with PVOD prior to the development of PH, which represents an interesting and provocative approach to treating these patients.
The round table discussion on off-label use of PAH therapy in patients with non-Group 1 PAH brought together experts in the management of PH related to left-sided heart disease, end-stage renal disease, sarcoidosis, sickle cell disease and other chronic hemolytic anemias, and chronic thromboembolic disease for a lively overview of how clinicians approach these challenging cases in their own practices. We were impressed by the often-overlapping approaches across centers, although clearly there were some differences in opinions.
This issue concludes with the 2023 PH Professional Network (PHPN) Symposium abstracts which allow all of us to review the science presented last fall at the conference. It is exciting to see the great work going on in PH clinical care.
We want to thank our contributing authors for their hard work in putting this issue together and for sharing their knowledge and experience with all of us. To our readers, we hope you enjoy this issue and learn things you can take back to your patients.