# Treatment options for pulmonary hypertension
The treatment of pulmonary hypertension differs based on the type and severity of the condition. A multidisciplinary team determines which approach is most suitable. Below are the main treatment strategies outlined.
Medications that work directly on the pulmonary vessels
Endothelin receptor antagonists
ProveniIncluded in official guidelines, or approved by EMA or FDA
These medications block a substance (endothelin) that causes the blood vessels around the lungs to constrict. By blocking this substance, the vessels can relax and the pressure in the lungs decreases.
The medications are usually given in tablet form. In many people, doctors see improvements in exercise capacity and less fatigue. Known side effects include fluid accumulation in the legs and feet, headaches, and nasal congestion. Some patients may develop elevated liver values that need to be monitored. This class of medications cannot be used in the early weeks of pregnancy.
Phosphodiesterase-5 inhibitors
ProveniIncluded in official guidelines, or approved by EMA or FDA
These medications (including sildenafil and tadalafil) work via a different mechanism: they cause smooth muscle tissue in the blood vessel wall to relax. This increases the diameter of the vessels and lowers the pressure.
They are given in tablet form. The goal is for patients to be able to exert themselves more without becoming short of breath quickly. Side effects include headaches, vision disturbances, increased oxygen consumption, and in rare cases severe drops in blood pressure. This group is also unsuitable for certain pregnancies.
Guanylate cyclase stimulators
ProveniIncluded in official guidelines, or approved by EMA or FDA
This is a newer class of medications (for example riociguat) that activates a different signaling pathway in the blood vessel wall. By stimulating that pathway, the vessels relax.
Riociguat is given in tablet form. Studies have shown that patients with certain types of pulmonary hypertension, particularly chronic thromboembolic forms, benefit from it. Side effects include dizziness, flushing and coughing. Riociguat cannot be used together with other vasodilators without restriction.
Prostanoids and prostacyclin receptor agonists
ProveniIncluded in official guidelines, or approved by EMA or FDA
These medications work via receptors on the blood vessel wall that regulate relaxation and fluid balance. Some are administered intravenously (for example epoprostenol), others as inhalation or in tablet form.
Intravenous variants require a permanent infusion system and continuous administration. Side effects can include facial flushing, abdominal pain and diarrhea. Particularly when stopped abruptly, cardiac symptoms and blood pressure changes can occur. Newer formulations (for example selexipag in tablet form) have a different side effect profile with fewer systemic effects.
Supportive therapies
Oxygen therapy
ProveniIncluded in official guidelines, or approved by EMA or FDA
When oxygen levels in the blood drop (especially during exertion or sleep), long-term oxygen therapy can help reduce the strain on the heart and lungs.
The oxygen is usually administered via a nasal or mouth piece. The goal is to keep blood oxygen above a certain threshold. Side effects include dryness of the nose and mouth, and logistical inconvenience from the equipment. Regular monitoring of oxygen levels determines whether adjustment is needed.
Diuretics (water-removing medications)
ProveniIncluded in official guidelines, or approved by EMA or FDA
When the right ventricle weakens under pressure, fluid can accumulate in the legs, abdomen and lungs. Diuretics help the body eliminate excess fluid.
They are given in tablet form. Side effects include electrolyte disturbances (salt and potassium), fatigue and sometimes dizziness with sudden movements. Regular blood tests are needed to prevent serious electrolyte disturbances.
Antithrombotic medications
ProveniIncluded in official guidelines, or approved by EMA or FDA
With certain types of pulmonary hypertension (particularly the chronic thromboembolic form), blood thinners may be needed to prevent blood clots.
These are given in tablet form. Side effects include increased bleeding risk (nose, gums, urine) and sometimes abdominal pain. Regular monitoring of coagulation values is essential.
Specialized procedures
Balloon pulmonary angioplasty (BPA)
ProveniIncluded in official guidelines, or approved by EMA or FDA
In patients with chronic thromboembolic pulmonary hypertension, deposits in the lung vessels can sometimes be removed via a catheter. For this, a balloon is carefully inflated in the narrowed vessel.
This is a clinical procedure performed in specialized centers. Complications can include bleeding, heart and breathing problems. Some patients undergo multiple sessions. Recent research (STOP-RIO study) examines the safety of stopping certain medications after successful BPA.
Atrial septostomy
ProveniIncluded in official guidelines, or approved by EMA or FDA
This is an emergency procedure for very severe pulmonary hypertension with heart failure. A small opening is deliberately made in the septum between the upper heart chambers, so that blood can bypass and the right ventricle does not have to work as hard.
This is a procedure with significant risk and is only used when other treatments do not help. Side effects include chronic oxygen loss and increased risk of heart rhythm problems. The procedure requires specific expertise.
Heart transplantation
ProveniIncluded in official guidelines, or approved by EMA or FDA
For patients whose pulmonary hypertension does not respond adequately to medications and whose heart is severely affected, heart transplantation may be an option.
This is a major surgical procedure with serious risks (infection, rejection, immune system suppression) and limitations (donor availability, lifelong medication use). It is reserved for very carefully selected patients in specialized centers.
Experimental and investigated approaches
Combination therapy and intensification
ResearchediPositive results in clinical studies, not yet standard treatment
Increasingly, doctors see that a combination of medications from different classes works better than one medication alone. Studies examine which combinations are optimal and how quickly intensification should occur.
The logic is that different mechanisms are addressed simultaneously. The risk of side effects increases; regular monitoring is essential.
Gene therapy and vascular genetic approach
ExperimentaliOngoing in study setting, outcome still unknown
New techniques such as CRISPR-based gene editing are exploring whether abnormalities in blood vessel cells can be corrected. This is currently taking place in laboratory and animal model research.
Many questions must be answered before this can be applied in patients.
Biomarkers and individualized treatment
ResearchediPositive results in clinical studies, not yet standard treatment
Research into plasma proteins and imaging markers (such as the ratio between right and left heart chambers) can help better predict who responds well to which treatment.
This could help with personalized treatment in the future, but is not yet standard practice.
Physical activity and rehabilitation
ResearchediPositive results in clinical studies, not yet standard treatment
Carefully guided physical activity and rehabilitation programs are being studied as a supplement to medications. The idea is that the heart can function better through targeted training.
This only happens under medical supervision. Too much exertion can be harmful; the so-called safe exertion limits must be carefully established.
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_This information never replaces a doctor's judgment. Always discuss your situation with your own healthcare provider._