The FDA has approved Orenitram (treprostinil) the first orally administered prostacyclin analogue for the treatment of pulmonary arterial hypertension (PAH).
Pulmonary hypertension (PH) is an elevated blood pressure through the lungs which can cause strain on the right side of the heart. It is difficult to diagnose and typically not a first thought by patients or physicians because the symptoms are nonspecific. Symptoms typically are shortness of breath with activity, exertional limitation and easy fatiguability. With continued strain on the right heart, swelling of the legs and/or increase in abdominal girth (belt becomes tighter) may occur.
Unlike systemic hypertension which can be measured with a blood pressure cuff around the arm, one cannot measure PH by placing a blood pressure cuff around the lung.
We often use an echocardiogram or ECHO (ultrasound of the heart) in order to look at the function of the right side of the heart, the side which pumps blood through the lungs. If this screen reveals an elevated pressure or stress on the heart, confirmation by direct measurement of the pressures in the lungs may be offered by a right heart catheterization.
The causes of pulmonary hypertension are categorized into five groups. Therapies are determined by which group the patient is categorized.
Because the symptoms are so nonspecific, diagnosis of PH may be missed for months or years.