For the first time, the FDA has approved the use of two medications as initial therapy for pulmonary arterial hypertension.  In response to the recently published AMBITION study results, the FDA has approved the use of Letairis (ambrisentan) and Adcirca (tadalafil) to be used as initial therapy in newly diagnosed patients with PAH.  Previously, many physicians would prescribe … [Read more...]

Treating Pulmonary Arterial Hypertension When There is Also Another Medical Problem, Such as Diastolic Heart Failure. We have received a variety of questions about how to treat pulmonary arterial hypertension when a second medical problem is present at the same time.  This is a very common situation in clinical practice.  I would like to focus on how to approach the … [Read more...]

Diuretics are medications that increase your urine production.  Most patients with PAH require diuretics at some point in their disease course.  This is due to abnormal hormone signals that are produced by your heart, lungs and kidneys that lead to inappropriate fluid retention.  In health, your salt and water balance in your body is carefully regulated.  If you eat a salty … [Read more...]

Sildenafil (Revatio) was approved in 2005 based on a 12-week study that showed improvements in six minute walk distance. In the initial study, there was not a clear difference between 20mg three times a day and higher doses. In a subsequent study that followed patients for years, the majority of patients increased their dose to 80mg three times daily and had lasting benefit. In … [Read more...]

FDA Approval of Opsumit This medicine, Opsumit (Macitentan), was approved in 2014 based on a very large long-term study of 742 patients.  250 were randomized to placebo (sugar pill), 250 were randomized to 3mg once daily of Opsumit, and 242 were randomized to 10mg once daily.  Patients were then followed and their rates of clinical deterioration were recorded.  Exercise … [Read more...]