Pulmonary hypertension (PH) is associated with increased pressure in the pulmonary artery. PH can have several causes and is often fatal. The prognosis for pulmonary hypertension is often worse than that for cancer. Scientific research into the disease remains important. Prof. Dr. Anton Vonk Noordegraaf of the VUmc explains this study.
Are there any medications for treating pulmonary hypertension?
Currently there are medicines that significantly extend and improve life and quality of life. We make small steps forward, but are still far from cure, which causes a significant proportion of patients to progress, despite the drugs. The current medication is mainly aimed at widening vessels to reduce blood pressure. The disease also includes remodeling, thickening and deformity of the blood vessel wall, which also causes blood pressure increase. With the current medication, this remodeling is still insufficiently counteracted. We are trying to get a breakthrough there.
What are the obstacles in doing scientific research?
It is extremely difficult to investigate in rare cases. This can only be if the patient population is concentrated and you work with foreign centers. We do that too, a global consortium has been established. Progression and progress can only be booked as pulmonary hypertension, and any other rare condition is treated centrally.
What do you expect in the long term for the treatment of pulmonary hypertension?
I expect it to be possible in the future to stabilize the disease in most patients. Healing will be very difficult. In addition, I hope that more donor lungs will be available so that my patients who retard therapy in the future can get a new lung in good time.
If you look more at short-term research, what findings are important?
In recent years, we have learned a lot about how to optimize the current medication in the treatment of this disease. In addition, we now know that rehabilitation helps patients in good shape. Each muscle contains a lot of iron. Pulmonary hypertension patients usually have a severe iron deficiency because their intestines do not absorb well. However, if you supplement the iron, it also strengthens the heart muscle. These are concrete solutions that you can already apply. Also, it is important that you do not allow the heart rate to increase too fast to prevent the heart from consuming too much oxygen. There are medicines to regulate that. Patients feel better and more objective and subjective. A point of attention is also the optimal care and guidance of the patients. Many patients suffer from anxiety disorders and depression. It requires a team approach to support these patients optimally.
Has progress been made in recent years?
Initially, we had no idea how and why that vessel wall so thickened; I think we have now fully understood the mechanism. The current path also provides points of action for drug development. Another point of concern is to strengthen and maintain the right ventricle to maintain high pressure in the pulmonary artery. The problem is often that the right ventricle becomes exhausted, resulting in heart failure. We understand better how these mechanisms work and can work to therapies that support the right heart room.
Are there any medications for treating pulmonary hypertension?
Currently there are medicines that significantly extend and improve life and quality of life. We make small steps forward, but are still far from cure, which causes a significant proportion of patients to progress, despite the drugs. The current medication is mainly aimed at widening vessels to reduce blood pressure. The disease also includes remodeling, thickening and deformity of the blood vessel wall, which also causes blood pressure increase. With the current medication, this remodeling is still insufficiently counteracted. We are trying to get a breakthrough there.
What are the obstacles in doing scientific research?
It is extremely difficult to investigate in rare cases. This can only be if the patient population is concentrated and you work with foreign centers. We do that too, a global consortium has been established. Progression and progress can only be booked as pulmonary hypertension, and any other rare condition is treated centrally.
What do you expect in the long term for the treatment of pulmonary hypertension?
I expect it to be possible in the future to stabilize the disease in most patients. Healing will be very difficult. In addition, I hope that more donor lungs will be available so that my patients who retard therapy in the future can get a new lung in good time.
If you look more at short-term research, what findings are important?
In recent years, we have learned a lot about how to optimize the current medication in the treatment of this disease. In addition, we now know that rehabilitation helps patients in good shape. Each muscle contains a lot of iron. Pulmonary hypertension patients usually have a severe iron deficiency because their intestines do not absorb well. However, if you supplement the iron, it also strengthens the heart muscle. These are concrete solutions that you can already apply. Also, it is important that you do not allow the heart rate to increase too fast to prevent the heart from consuming too much oxygen. There are medicines to regulate that. Patients feel better and more objective and subjective. A point of attention is also the optimal care and guidance of the patients. Many patients suffer from anxiety disorders and depression. It requires a team approach to support these patients optimally.
Has progress been made in recent years?
Initially, we had no idea how and why that vessel wall so thickened; I think we have now fully understood the mechanism. The current path also provides points of action for drug development. Another point of concern is to strengthen and maintain the right ventricle to maintain high pressure in the pulmonary artery. The problem is often that the right ventricle becomes exhausted, resulting in heart failure. We understand better how these mechanisms work and can work to therapies that support the right heart room.