Patients with primary pulmonary hypertension can be managed based on the severity of their condition and the response to certain drug tests.
Criteria for the diagnosis of primary pulmonary hypertension:
(1) mean pulmonary artery pressure > 25 mm Hg at rest or > 30 mm Hg during exercise
(2) exclusion of left sided cardiac valvular disease, myocardial disease, congenital heart disease, important respiratory disease, important connective tissue disease and thromboembolic diseases
All patients are anticoagulated.
Patient are then divided based on the response to vasoreactivity by challenge test with:
(1) inhaled nitric oxide
(2) intravenous epoprostenol
(3) intravenous adenosine
Response to Vasoreactivity Challenge
Response to Oral Calcium Channel Blocker
NYHA Class
Therapy in addition to Anticoagulation
minimal to moderate fall in pulmonary vascular resistance
NA
1 or 2
possible oral calcium channel blocker therapy
NA
3 or 4
epoprostenol; consider
transplantation
marked fall in pulmonary vascular resistance
response
oral calcium channel blocker therapy
no response
1 or 2
no additional therapy
no response
3 or 4
epoprostenol; consider
transplantation
after Figure 2, page 116
where:
• NA = not applicable
• NYHA = New York Heart Association Class
• oral calcium channel blockers include nifedipine and diltiazem
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