About this Research Topic
The management of patients with congenital heart disease (CHD) and
pulmonary arterial hypertension (PAH) has changed dramatically with the development of targeted therapy with selective pulmonary vasodilators. It is important to develop evidence-based guidelines for the management of these patients. However, there is not enough evidence regarding the operability of CHD with PAH. An atrial septal defect is a simple CHD and can be closed surgically or using a catheter interventional technique relatively easily. However, because heart murmur is not loud in patients with ASD, ASD is sometimes not diagnosed until PAH is advanced. Controversy exists if ASD can be closed in patients with severe PAH or not. Furthermore, the role of pulmonary vasodilators in the management of PAH associated with ASD has not been established.
The current consensus about operability is a pulmonary resistance of <8 Wood Units (WU)·m2. However, these criteria may be too conservative. The criteria determining operability in patients with PAH associated with CHD in the era of pulmonary vasodilators should be newly established. The goal of this project is to raise questions regarding the current criteria of operability of ASD with PAH and show some evidence that the current criteria may be expanded. In some countries, ASD in patients with severe PAH had been closed. In some patients
with severe PAH, pulmonary vasodilators are administered first and several months later ASD is closed. This treat-and-repair strategy is getting attention. The long-term results of these strategies remain unclear and should be clarified.
In this Research Topic, we welcome, but are not limited to manuscripts exploring the following points:
1) Current data regarding the long-term prognosis of patients with ASD and severe PAH including Eisenmenger syndrome
2) Review of the current consensus about the operability of ASD with PAH
3) In some countries, the criteria is a pulmonary resistance of <8 Wood Units (WU)·m2 had not been followed. What impact does it have?
4) Review of histological changes in the lung in patients with ASD and describe any unique points in ASD
5) Long-term results when ASD in patients with severe PAH had been closed surgically or by catheter intervention
6) Long-term results when in some patients with severe PAH, pulmonary vasodilators are administered first and several months later ASD is closed.
pulmonary arterial hypertension (PAH) has changed dramatically with the development of targeted therapy with selective pulmonary vasodilators. It is important to develop evidence-based guidelines for the management of these patients. However, there is not enough evidence regarding the operability of CHD with PAH. An atrial septal defect is a simple CHD and can be closed surgically or using a catheter interventional technique relatively easily. However, because heart murmur is not loud in patients with ASD, ASD is sometimes not diagnosed until PAH is advanced. Controversy exists if ASD can be closed in patients with severe PAH or not. Furthermore, the role of pulmonary vasodilators in the management of PAH associated with ASD has not been established.
The current consensus about operability is a pulmonary resistance of <8 Wood Units (WU)·m2. However, these criteria may be too conservative. The criteria determining operability in patients with PAH associated with CHD in the era of pulmonary vasodilators should be newly established. The goal of this project is to raise questions regarding the current criteria of operability of ASD with PAH and show some evidence that the current criteria may be expanded. In some countries, ASD in patients with severe PAH had been closed. In some patients
with severe PAH, pulmonary vasodilators are administered first and several months later ASD is closed. This treat-and-repair strategy is getting attention. The long-term results of these strategies remain unclear and should be clarified.
In this Research Topic, we welcome, but are not limited to manuscripts exploring the following points:
1) Current data regarding the long-term prognosis of patients with ASD and severe PAH including Eisenmenger syndrome
2) Review of the current consensus about the operability of ASD with PAH
3) In some countries, the criteria is a pulmonary resistance of <8 Wood Units (WU)·m2 had not been followed. What impact does it have?
4) Review of histological changes in the lung in patients with ASD and describe any unique points in ASD
5) Long-term results when ASD in patients with severe PAH had been closed surgically or by catheter intervention
6) Long-term results when in some patients with severe PAH, pulmonary vasodilators are administered first and several months later ASD is closed.
Keywords: Pulmonary vasodilator, Eisenmenger syndrome, Pulmonary hypertension, Atrial septal defect, Operability, Pulmonary resistance, Congenital heart disease
Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.
