Leading pulmonary hypertension expert reviews PAH therapy and trends. How to select best treatment for pulmonary hypertension? What is new in treatment of pulmonary arterial hypertension? Dr. Anton Titov, MD. Treatment of pulmonary arterial hypertension has come a long way since I got involved. Dr. Aaron Waxman, MD. I first got involved in therapy of pulmonary arterial hypertension a long time ago. Then there was only one medication. Or two medications. There were calcium channel blockers. They are ineffective for the vast majority of patients with pulmonary arterial hypertension. There was epoprostenol, which was called Flolan at the time. It is an intravenous therapy. Prostacyclin was the other medication. Prostacyclin had a big impact on patients, but it was a very complicated therapy. Dr. Aaron Waxman, MD. It involved intravenous continuous infusion. Injection had to be via external pumps and Hickman catheters. Over the past 20 years the focus in pulmonary arterial hypertension treatment has been on three main pathways. Endothelin pathway, nitric oxide pathway, and prostacyclin pathway. There are several medications developed for pulmonary arterial hypertension. Dr. Aaron Waxman, MD. They are intravenous injections and oral medications. There is even inhaled medication. But none of the medications can cure pulmonary arterial hypertension. New medications are all effective in pulmonary arterial hypertension to a certain extent. They have had a tremendous impact on survival and functional status of patients with pulmonary arterial hypertension. New medications improved quality of life. But there is still tremendous room for development of effective pulmonary arterial hypertension therapy. More and more the focus is now on metabolic modulators. These medications change mitochondrial function. There are antiproliferative medication candidates. There are even chemotherapeutic medications. They are being re-purposed from cancer treatment. Also there are anti-inflammatory targets in pulmonary arterial hypertension. It is a complicated method of therapy. Dr. Aaron Waxman, MD. We are moving much more into the realm of successful therapy of pulmonary arterial hypertension. We are starting to treat pulmonary arterial hypertension like a metabolic neoplastic disease. It is more than a vasoconstrictor disease. Most of the focus previously has been on vascular constriction aspects of pulmonary arterial hypertension. There is a patient who has been diagnosed with pulmonary arterial hypertension. Dr. Aaron Waxman, MD. Let’s suppose that the diagnosis has been established. Are there particular stages of therapy of pulmonary arterial hypertension? Dr. Anton Titov, MD. Are there lines of therapy in pulmonary arterial hypertension, similarly to the cancer treatment? Dr. Anton Titov, MD. What is a typical progression of treatment for a patient with pulmonary arterial hypertension? Dr. Anton Titov, MD. We grade a severity of disease in a patient with pulmonary arterial hypertension. We determine a severity grade scale much like we do in heart failure. We use the WHO functional class approach. Dr. Aaron Waxman, MD. Over the past two to three years it has become the standard of care for pulmonary arterial hypertension. We use a combination therapy. We would use a combination treatment for most other complicated disease. These medications for pulmonary arterial hypertension tend to be quite expensive. There has been a reluctance over the past years to treat pulmonary arterial hypertension purely because of expense. Now we have clinical trial data that show a clear benefit in pulmonary arterial hypertension treatment. It is best to use a combination therapy with several medications. It depends on how sick a patient with pulmonary arterial hypertension might be. By how sick I mean how short of breath the patient is. How limited is a patient in physical ability? Dr. Anton Titov, MD. Are they having problems with heart failure? Dr. Anton Titov, MD. Dr. Aaron Waxman, MD. We might start with just oral therapy for pulmonary arterial hypertension. Then we can use a combination of two medications. Usually our treatment is a phosphodiesterase 5 inhibitor in combination with an endothelin antagonist. Patients with pulmonary arterial hypertension progress in severity. Then we start to think more and more about prostacyclins. But the truth is that. Prostacyclins are still our best therapies for pulmonary arterial hypertension. Probably if we use them earlier in disease, we'd get even better results. It is a moving target how we treat patients with pulmonary arterial hypertension. But it is good. Dr. Aaron Waxman, MD. It is becoming a more complex targeting of pulmonary arterial hypertension disease. We use a more precise therapeutic approach to pulmonary arterial hypertension. That leads to better outcomes for patients.