The past two decades have witnessed important progress in the treatment of
primary pulmonary disease (PPH). Although significant improvements in quali
ty of life and survival rate have been obtained with prostacyclin therapy,
and better perspectives are now offered with atrial septostomy and lung tra
nsplantation, conventional therapy has also evolved. Patients are now more
adequately counseled regarding how to remain physically active while avoidi
ng exercise-induced anginal pain and syncope. Invasive and potentially risk
y medical procedures have been restricted in favor of noninvasive and funct
ional evaluations whenever possible.