Pulmonary Hypertension: from Bench to Bedside

Pulmonary hypertension (PH) is a hemodynamic condition that underlies many diseases. Although PH is defined by strict hemodynamic criteria, patients with PH are very heterogeneous. This makes screening and diagnosis very difficult, presenting an unmet need. Targeted therapies, which improve exercise capacity and survival, are available for patients with pulmonary arterial hypertension and chronic thromboembolic hypertension. However, there is still space for improvement for long-term prognosis. Currently, there are no worldwide approved targeted therapies for patients who suffer from other forms of PH (PH due to left heart disease, PH due to lung diseases, PH due to multifactorial or unclear mechanisms). The 6th World Symposium on Pulmonary Hypertension in 2018 summarized recent evidence in the field, and the guidelines for diagnosis and therapy for pulmonary hypertension are to be expected in 2022.

The state of the art and research perspectives of pathology and pathobiology of pulmonary hypertension were published in 2019 by prominent experts in the field. One of the most exciting challenges is translating pre-clinical findings to clinical studies for a rare disease like PH. Because animal models do not recapitulate the full spectrum of human disease, it is of great interest to use human samples like tissues from explanted lungs or blood samples. In order to provide individualized therapies, rigorous phenotyping, endotyping and genotyping are essential. Biomarkers that guide diagnosis, therapeutic decisions and prognosis would be ideal tools to achieve better patient care.

Dysfunction of the pulmonary endothelium, abnormal accumulation of smooth muscle cells and adventitial fibroblast as well as dysregulation of the immune system, are pathophysiological processes on a cellular level in PH that warrant further investigation. On a molecular level, ion channel abnormalities, transcription signaling dysregulations, epigenetic disturbances and metabolic changes are the most promising topics with translational potential. We seek high-quality papers that would fill the gap between bench and bedside. Original articles that include well-characterized real-world patient collectives are desired.
We want to ask contributors to submit papers that address the following themes:
- translational approach for new molecules targeting PH pathways
- translational approach for repurposed molecules targeting PH pathways
- new treatment options for PH groups II, III, V
- biomarkers of diagnosis for a particular type of PH
- biomarkers that distinguish "responders" from "non-responders" to targeted PH therapies