1. Warfarin therapy should be undertaken if deemed safe. The general-ly
   accepted INR range is 1.5-2.5. Diuretics, digoxin, and oxygen are
   utilized on an individual basis.
   
2. Right-heart catheterization is essential in determining initial and
   sometimes subsequent therapy. This, together with WHO classification,
   echocardiographic data and exercise testing are used in treatment
   decisions. The rate at which symptoms are progressing may play a
   role in the level of aggressiveness with therapy. The terms IIIa and IIIb
   denote early, stable class III patients and advanced class III patients,
   respectively.
   
3. Unresponsive class I-II patients are individualized; one option is
   enrollment in clinical research trials as in class IIIa. For vasoreactive
   patients, calcium channel blockers (CCB) alone may be appropriate
   when the vasodilator response is exceptional.
   
4. In those who respond to CCB, but suboptimally, or who respond but
   clinically worsen, oral bosentan (Tracleer) should be strongly consid-ered.
   This drug is approved for WHO class III-IV patients. There is no
   clear consensus on the use of this drug as it relates to presence or
   absence of vasodilator response. Bosentan (Tracleer) should not be
   used in setting of liver disease.
   
5. Treprostinil (Remodulin) is an investigational subcutaneous prostacy-clin
   analogue (approvable letter for class II-IV PAH), and iloprost (not
   available in U.S.) is an investigational inhaled prostacyclin analogue.
   Other investigational agents may be considered in stable class II-III
   patients in the setting of clinical research trials.
6. Epoprostenol (Flolan) is the FDA-approved intravenous prostacyclin
   for class III-IV patients and is the most effective form of therapy in
   these individuals. Bosentan is appropriate for most class IIIa patients
   prior to considering epoprostenol, but in class IIIb-IV patients,
   epoprostenol is preferred. The distinction between class IIIb and class
   IV is essentially arbitrary as these patients are generally handled in
   the same manner. The relative roles of bosentan and treprostinil are
   not well defined and the latter awaits final approval. Combined therapy
   with the addition of bosentan and/or sildenafil could be considered
   but would be investigational; clinical trials are ongoing or planned.
   
7. Very few centers have extensive experience with atrial septostomy.
   When utilized, this is intended to serve as a bridge to transplantation.
   The timing of lung transplant referral is individualized at different cen-ters.
   This depends in part upon the waiting time at the listing institution.

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