FAQ’s: Implanted Remodulin Pump, Right Heart Catheterization, PAH & Pregnancy

Your Questions Answered.

Our readers have submitted some great questions.  We value your questions and encourage you to continue to tell us about your interests and questions.

What is happening with the implanted Remodulin Pump?

The FDA recently announced they are not ready to approve the implanted pump system for the delivery of continuously infused Remodulin.  They are asking that more work be done prior to approving the system.  Many patients across the United States have been eagerly awaiting approval of the implanted system.  Medtronic and United Therapeutics continue to work to bring this important treatment to patients.  No timeline has been released.

Is a right heart catheterization necessary to diagnose pulmonary arterial hypertension?

Internal anatomy of the pulmonary arteriesThe emphatic answer is absolutely.  If your physician wants to diagnose you as having pulmonary arterial hypertension without performing a right heart catheterization then you would be best served by seeking a more expert opinion.

While an echocardiogram may point to the diagnosis, it is never enough to make a firm diagnosis or initiate treatment.

The Pulmonary Hypertension Association has developed a center of excellence label “Comprehensive Care Center” that has been awarded to more than 30 programs across the country.  These programs have a demonstrated track record of outstanding care in all aspects of PAH.

We received a question about PAH and pregnancy

Pregnancy is very dangerous for women with PAH.  Depending on the severity of a woman’s PAH, mortality may be as high as 50%.  As a woman moves through the first trimester of pregnancy the blood volume increases by about 50%, peaking in the third trimester.  In patients with PAH, the weakened right ventricle is unable to accommodate the increased blood volume and may further decline.  This results in worsening right heart failure.  A second period of great risk involves delivery.

PAH patient storyLastly, in the first week after delivery there is a process where edema fluid moves back into the blood vessels and results in rapid increases in blood volume.  This can precipitate abrupt right heart failure.  Adding to the risk of pregnancy is that all endothelin receptor antagonists are 100% contraindicated in pregnancy due to risk of birth defects.  Any woman with PAH who is pregnant should immediately be seen by a high-risk obstetrician and a PAH expert at a center of excellence.