The cost of Pulmonary Arterial Hypertension medications is staggering.  The least expensive medication  (generic sildenafil) costs $50 per month for 90 tablets.  If you prefer to have the branded version and you don’t have any insurance expect to pay more than $3,000 per month.  Insurance companies are very happy to pay for generic sildenafil but increasingly restrict Adcirca … [Read more...]

New Study Comparing Medication Combinations in Treatment of Pulmonary Arterial Hypertension Actelion Pharmaceutics has undertaken an important new study that compares two medications versus three medications in patients that are newly diagnosed with PAH. Currently we can say with confidence that most patients do better when treated initially with an endothelin receptor … [Read more...]

Swelling of the Scrotum with Pulmonary Arterial Hypertension. We recently received a great question about scrotal edema (swelling of scrotum and often the penis as well).  This is a very common problem for men with PAH who are retaining large amounts of fluid. Not only is this problem very uncomfortable but also it can make urinating a challenge. When patients have so much … [Read more...]

In 1995 epoprostenol, a continuously infused prostanoid medication, was approved for the treatment of PAH. Although this medicine worked to decrease pulmonary artery pressures, there were many risks with this therapy in congenital heart disease (CHD) patients.  Since an intravenous catheter was required, there was a small but real risk of blood stream infection.  There was also … [Read more...]

The question of whether anticoagulation (thinning of blood with medications) should be used in the treatment of pulmonary hypertension dates back more than 35 years.  Very early observations of small blood clots in the lungs of PAH patients propelled interest in using warfarin (our oldest blood thinner). In the 1980’s and 1990’s several small studies were completed that … [Read more...]