Sean Studer, MD, MSc, FCCP, Head of Medical Affairs at the US Pulmonary Hypertension Group, at Johnson & Johnson, discusses the changing treatment landscape of pulmonary arterial hypertension (PAH), including the approval of Opsynvi (macitentan plus tadalafil).
PAH affects the heart and lungs. It is characterized by hypertension in the pulmonary artery. Symptoms include:
- Shortness of breath during exercise
- Fainting spells
- Dizziness
- Swelling of the ankles or legs
- Chest pain
- Racing pulse
Some cases of PAH are due to genetic changes in the BMPR2 gene, however most cases of PAH occur in individuals with no family history of the disorder. This is also known as idiopathic PAH. PAH can also occur secondary to underlying conditions such as connective tissue diseases, HIV infection, chronic hemolytic anemia, and congenital heart disease. PAH can also be induced by certain drugs and toxins.
As Dr. Studer explains, the combination therapy of macitentan and tadalafil provides an innovative approach to PAH treatment. Typically, treatment consists of a single medication initially, with the addition of other treatment options as the disease progresses. Because patients often face delayed diagnosis, irreversible damage has often been done and focus is placed on symptom management and preventing further damage. The use of macitentan and tadalafil together adheres better to guideline recommendations of initial combination therapy, giving better results initially and prolonging disease progression.
To learn more about PAH and other rare lung diseases, visit https://checkrare.com/diseases/lung-diseases/
