A new drug is poised to enter the PAH market and offers great promise for patients. This new drug works in a completely different way from all the currently approved medications. Sotatercept is an injection (shot) that is given subcutaneously (under the skin) every 3 weeks. The medication acts by blocking activin. In PAH there are good signals and bad signals within the blood … [Read more...]
Aerovate and Inhaled Imatinib
Imatinib is a tyrosine kinase inhibitor that has been on the market for more than 20 years to treat a type of leukemia. It acts by turning off tyrosine kinases. You can think of tyrosine kinases as critical switches in cells that turn on and off critical pathways. Too much of an “on” signal leads to dysregulated cell proliferation and growth—such as seen … [Read more...]
Sotatercept for Pulmonary Arterial Hypertension
For the past 30 years, all our pulmonary arterial hypertension medications were described as vasodilators: First prostanoids (epoprostenol), then endothelin receptor antagonists (bosentan, ambrisentan, macitentan) and PDE5 inhibitors (sildenafil and tadalafil). These medications work through different mechanism but all lead to the vasodilation or … [Read more...]
Pulmonary Hypertension and Interstitial Lung Disease
Pulmonary hypertension (elevated pressure within the blood vessels of the lungs) is organized into five groups: Group 1: Pulmonary arterial hypertension (PAH) Group 2: Pulmonary venous hypertension (due to problems on the left side of the heart) Group 3: Pulmonary hypertension due to lung disease Group 4: Pulmonary hypertension due … [Read more...]
Lung Transplantation with PAH
For Pulmonary Arterial Hypertension (PAH) patients who remain very short of breath despite maximal medical therapy, lung transplantation may be an option. Maximal medical therapy usually means continuously infused therapy (Remodulin, Flolan, Veletri) and multiple oral medications. First, it is important to know that lung transplantation is not a … [Read more...]
