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Midwest Pediatric Cardiology Nurses Association


Pulmonary Hypertension
Submitted by Pegi Shaner, RN, MSN, CPNP

 “The 6th Annual Pulmonary Hypertension Symposium” was held in the Fall of 2005 in Baltimore, Maryland. The keynote speaker was Congressman Tom Lantes (CA-12) whose granddaughter was struck with pulmonary hypertension (PH) several years ago at the age of eighteen. His passionate pleas for government funding to help understand and conquer this disease were apparent as he talked about introducing the PH Research Act to congress in June 2005. He hopes this act will fund groundbreaking medical research dedicated to finding treatments and possibly a cure for PH.

The PH Research Act will help convert hope into something more concrete. The legislation authorized 250 million dollars over five years to fund PH research and fulfill three key objectives. First, it expands PH research at the National Heart, Lung, Blood Institute (NHLBI). Second, it establishes research and education efforts for both health professionals and the general public. Third, it establishes a data system and clearinghouse at the NHLBI.

In addition to this wonderful news, there are also several new drugs and therapies becoming available to patients with PH. Added to the traditional therapies of oxygen, anticoagulants, calcium channel blockers and prostacycline, patients now have the opportunity to try other prostanoids such as Treprostinil (Remodulin) which can be delivered through a central line similar to Prostacycline or given subcutaneously. Another prostanoid is Iloprost; also known as Ventavis, which is the inhaled medication for PH. Another class of medications is the Endothelin Antagonists which Bosentan (Tracleer) can be given as a pill. Sildenafil, also known as Viagra or Ravatio, is a Phosphodiesterase inhibitor that can be given orally. At St. Louis Children’s Hospital, we utilize all the above therapies to help our patients control their pulmonary hypertension.

The impact of all the new therapies is encouraging to patients and their families as well as to medical professionals who treat them. The new drugs and therapies prevent lung and heart/lung transplantation from occurring for many years!

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© 2006 MPCNA - Midwest Pediatric Cardiology Nurses Association