A range of underlying conditions can lead to Pulmonary Hypertension (PH). Accordingly, PH is classified into 5 clinical subgroups: pulmonary arterial hypertension (PAH, Group 1), PH due to left heart disease (PH-LHD, Group 2), PH due to hypoxia or chronic lung disease (PH-CLD, Group 3), Chronic thromboembolic pulmonary hypertension (CTEPH, Group 4), and PH with multifactorial and/or unclear mechanisms (Group 5). So far PH remains to associate with a high morbidity and mortality, although significant advances in both the underlying mechanisms and targeting medications have been made. PAH is characterized beyond vessels pulmonary contraction but vascular remodeling. The current targeted drugs are mostly pulmonary vascular dilators for short-term symptom improvement but with poor long-term outcomes. In addition, specific medication for Group 2 and Group 3 is still lacking so far. Therefore, further investigations for better understanding of PH mechanism leading to breakthrough therapy targets are called for.
Many newly discovered mechanisms of different types of PH have been investigated in recent years. For instance, Gene mutation of type 2 Bone Morphogenetic Protein Receptor (BMPR2) is frequently reported both in hereditary and idiopathic patients so that it has been believed as one of the common gene genovariation among PAH. Sotatercept, a novel fusion protein, binding activins and growth differentiation factors to restore balance between growth-pomoting and growth-inhibiting signals in BMP pathways, has been proved to further improve the long-term outcomes of PAH. Neurofibromatosis type 1 (NF1), also known as von Recklinghausen disease, associated with interstitial lung disease, is newly discovered that there is an association with severe PH without parenchymal lung disease. The chemokines and cytokines implicated that, from a biomarker platform to immunity related adverse remodeling in PH, targeting inflammatory/immune mechanisms are promising. Genetic and metabolic abnormalities are inextricably linked to dysregulated immunity and adverse remodeling in the pulmonary arteries.
New targeting medication beyond current 3 typical pathways are merging. In particular, the novel drugs aimed for reversing/inhabiting pulmonary vascular remolding. Furthermore, the breakthrough therapies are encouraged for other types of PH beside of group 1 PAH. For instance, Group 2 and Group 3 are the types leading to most PH but no special pulmonary vascular targeted medications recommended in clinic, in addition to the primary cause. New biomarkers or/and risks from basic origination are demanded as well, which will guide future clinic for anticipation of risk stratification and long-term outcomes with type differentiation.
In this Research Topic, we would like to create a forum for the new discoveries on the mechanisms of PH, which drive for more novel treatments for better outcomes based on current therapies. New genetic, cellular and physiological mechanisms linking pulmonary vascular remolding, and following clinical consequences are encouraged. Breakthrough therapies base on experimental studies for Group 2 and Group 3 are welcomed as well.
We welcome submissions of the following subtopics, but not limited to:
1) Investigation on the mechanisms of different PH models.
2) New or race specific gene screening in PH with congenital heart diseases, IPAH, HPAH etc.
3) Biomarkers for severity and risk assessment for PH.
4) Novel treatment targeting pulmonary vascular remolding.
5) Breakthrough treatments for Group 2 or group 3 PH.
6) Development in non-invasive techniques including CEPT, Cardiac MRI, Pet CT, V/Q scan etc. in PH or right heart evaluation.
7) Pulmonary vascular microcirculation and small airway studies in PH.
A range of underlying conditions can lead to Pulmonary Hypertension (PH). Accordingly, PH is classified into 5 clinical subgroups: pulmonary arterial hypertension (PAH, Group 1), PH due to left heart disease (PH-LHD, Group 2), PH due to hypoxia or chronic lung disease (PH-CLD, Group 3), Chronic thromboembolic pulmonary hypertension (CTEPH, Group 4), and PH with multifactorial and/or unclear mechanisms (Group 5). So far PH remains to associate with a high morbidity and mortality, although significant advances in both the underlying mechanisms and targeting medications have been made. PAH is characterized beyond vessels pulmonary contraction but vascular remodeling. The current targeted drugs are mostly pulmonary vascular dilators for short-term symptom improvement but with poor long-term outcomes. In addition, specific medication for Group 2 and Group 3 is still lacking so far. Therefore, further investigations for better understanding of PH mechanism leading to breakthrough therapy targets are called for.
Many newly discovered mechanisms of different types of PH have been investigated in recent years. For instance, Gene mutation of type 2 Bone Morphogenetic Protein Receptor (BMPR2) is frequently reported both in hereditary and idiopathic patients so that it has been believed as one of the common gene genovariation among PAH. Sotatercept, a novel fusion protein, binding activins and growth differentiation factors to restore balance between growth-pomoting and growth-inhibiting signals in BMP pathways, has been proved to further improve the long-term outcomes of PAH. Neurofibromatosis type 1 (NF1), also known as von Recklinghausen disease, associated with interstitial lung disease, is newly discovered that there is an association with severe PH without parenchymal lung disease. The chemokines and cytokines implicated that, from a biomarker platform to immunity related adverse remodeling in PH, targeting inflammatory/immune mechanisms are promising. Genetic and metabolic abnormalities are inextricably linked to dysregulated immunity and adverse remodeling in the pulmonary arteries.
New targeting medication beyond current 3 typical pathways are merging. In particular, the novel drugs aimed for reversing/inhabiting pulmonary vascular remolding. Furthermore, the breakthrough therapies are encouraged for other types of PH beside of group 1 PAH. For instance, Group 2 and Group 3 are the types leading to most PH but no special pulmonary vascular targeted medications recommended in clinic, in addition to the primary cause. New biomarkers or/and risks from basic origination are demanded as well, which will guide future clinic for anticipation of risk stratification and long-term outcomes with type differentiation.
In this Research Topic, we would like to create a forum for the new discoveries on the mechanisms of PH, which drive for more novel treatments for better outcomes based on current therapies. New genetic, cellular and physiological mechanisms linking pulmonary vascular remolding, and following clinical consequences are encouraged. Breakthrough therapies base on experimental studies for Group 2 and Group 3 are welcomed as well.
We welcome submissions of the following subtopics, but not limited to:
1) Investigation on the mechanisms of different PH models.
2) New or race specific gene screening in PH with congenital heart diseases, IPAH, HPAH etc.
3) Biomarkers for severity and risk assessment for PH.
4) Novel treatment targeting pulmonary vascular remolding.
5) Breakthrough treatments for Group 2 or group 3 PH.
6) Development in non-invasive techniques including CEPT, Cardiac MRI, Pet CT, V/Q scan etc. in PH or right heart evaluation.
7) Pulmonary vascular microcirculation and small airway studies in PH.
